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A04班

辻　省次　(東京大学・医学部附属病院・神経内科) Wakabayashi K, Engelender S, Yoshimoto M, Tsuji S, Ross CA, Takahashi H: Synphilin-1 is present in Lewy bodies in Parkinson’s disease. Ann Neurol 47: 521-523, 2000 Yamada M, Piao Y-S, Toyoshima Y, Tsuji S, Takahashi H: Ubiquitinated filamentous inclusions in cerebellar dentate nucleus neurons in dentatorubral-pallidoluysian atrophy contain expanded polyglutamine stretches. Acta Neuropathol 99: 615-618, 2000 Koide T, Yamada M, Takahashi T, Igarashi S, Masuko M, Furukawa T, Kuroha T, Koike T, Sato M, Tanaka R, Tsuji S, Takahashi H: Cyclosporine A-associated fatal central nervous system angiopathy in a bone marrow transplant recipient: an autopsy case. Acta Neuropathol 99: 680-684, 2000 Shibasaki Y, Tanaka H, Iwabuchi K, Kawasaki S, Kondo H, Uekawa K, Ueda M, Kamiya T, Katayama Y, Nakamura A, Takashima H, Nakagawa M, Masuda M, Utsumi H, Nakamuro T, Tada K, Kurohara K, Inoue K, Koike F, Sakai T, Tsuji S, Kobayashi H: Linkage of autosomal recessive hereditary spastic paraplegia with mental impairment and thin corpus callosum to chromosome 15q13-15. Ann Neurol 48: 108-112, 2000 Yamashita I, Sasaki H, Yabe I, Fukazawa T, Nogoshi S, Komeichi K, Takada A, Shiraishi K, Takiyama Y, Nishizawa M, Kaneko J, Tanaka H, Tsuji S, Tashiro K: A novel locus for dominant cerebellar ataxia (SCA14) maps to a 10.2-cM interval flanked by D19S206 and D19S605 on chromosome 19q13.4-qter. Ann Neurol 48: 156-163, 2000 Maruyama M, Ikeuchi T, Saito M, Ishikawa A, Yuasa T, Tanaka H, Hayashi S, Wakabayashi K, Takahashi H, Tsuji S: Novel mutations, pseudo-dominant inheritance, and possible familial affects in patients with autosomal recessive juvenile parkinsonism. Ann Neurol 48: 245-250, 2000 Takano H, Koike R, Onodera O, Tsuji S.: Mutational analysis of X-linked adrenoleukodystrophy gene. Cell Biochem Biophys 32: 177-185, 2000 Shimohata T, Nakajima T, Yamada M, Uchida C, Onodera O, Naruse S, Kimura T, Koide R, Nozaki K, Sano Y, Ishiguro H, Sakoe K, Ooshima T, Sato A, Ikeuchi T, Oyake M, Sato T, Aoyagi Y, Hozumi I, Nagatsu T, Takiyama Y, Nishizawa M, Goto J, Kanazawa I, Davidson I, Tanese N, Takahashi H, Tsuji S: Expanded polyglutamine stretches associated with CAG repeat diseases interact with TAFII130, interfering with CREB-dependent transcription. Nat Genet 26: 29-35, 2000 Tsuchiya K, Shintani S, Nakabayashi H, Kikugawa K, Nakano R, Haga C, Nakano I, Ikeda K, Tsuji S: Familial amyotrophic lateral sclerosis with onset in bulbar sign, benign clinical course, and Bunina bodies: a clinical, genetic, and pathological study of a Japanese family. Acta Neuropathol 100: 603-607, 2000 Hayashi S, Wakabayashi L, Ishikawa A, Nagai H, Saito M, Maruyama M, Takahashi T, Ozawa T, Tsuji S, Takahashi H: An autopsy case of autosomal-recessive juvenile parkinsonism with a homozygous exon 4 deletion in the parkin gene. Mov Disord 15: 884-888, 2000 Shimohata T, Onodera O, Tsuji S: Interaction of expanded polyglutamine stretches with nuclear transcription factors leads to aberrant transcriptional regulation in polyglutamine diseases. Neuropathology 20: 326-333, 2000 Yamada M, Wood JD, Shimohata T, Hayashi S, Tsuji S, Ross CA, Takahashi H: Widespread occurrence of intranuclear atrophin-1 accumulation in the central nervous system neurons of patients with dentatorubral-pallidoluysian atrophy. Ann Neurol 49: 14-23, 2001 Gaspar C, Lopes-Cendes I, Hayes S, Goto J, Arvidsson K, Dias A, Silveira I, Maciel P, Coutinho P, Lima M, Zhou Y-X, Soong B-W, Watanabe M, Giunti P, Stevanin G, Riess O, Sasaki H, Hsieh M, Nicholson GA, Brunt E, Higgins JJ, Lauritzen M, Tranebjaerg L, Volpini V, Wood N, Ranum L, Tsuji S, Brice A, Sequeiros J, Rouleau GA: Ancestral origins of the Machado-Joseph disease mutation: A worldwide haplotype study. Am J Hum Genet 68: 523-528, 2001 Yamada M, Hayashi S, Tsuji S, Takahashi H: Involvement of the cerebral cortex and autonomic ganglia in Machado-Joseph disease. Acta Neuropathol 101: 140-144, 2001 Kawachi I, Nozaki H, Watanabe M, Sato A, Tsuji S: Spontaneous spinal cord herniation. Neurology 56: 977, 2001 Shimohata T, Onodera O, Tsuji S: Expanded polyglutamine stretches lead to aberrant transcriptional regulation in polyglutamine diseases. Hum Cell 14: 17-25, 2001 Nakamura K, Jeong SE, Uchihara T, Anno M, Nagashima K, Nagashima T, Ikeda S, Tsuji S, Kanazawa I: SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein. Hum Mol Genet 10: 1441-1448, 2001 Sekine M, Nagata H, Tsuji S, Hirai Y, Fujimoto S, Hata M, Kobayashi I, Fujii T, Nagata I, Ushijima K, Obata K, Suzuki, M, Yoshinaga M, Umesaki N, Sato S, Enomoto T, Motoyama S, Tanaka K: The Japanese familial ovarian cancer study group: Localization of a novel susceptibility gene for familial ovarian cancer to chromosome 3p22-p25. Hum Mol Genet 10: 1421-1429, 2001 Date H, Onodera O, Tanaka H, Iwabuchi K, Uekawa K, Igarashi S, Koike R, Hiroi T, Yuasa T, Awaya Y, Sakai T, Takahashi T, Nagatomo H, Sekijima Y, Kawachi I, Takiyama Y, Nishizawa M, Fukuhara N, SaitoK, Sugano S, Tsuji S: Early-onset ataxia with ocular motor apraxia and hypoalbuminemia is caused by mutations in a new HIT superfamily gene. Nat Genet 29: 184-188, 2001 Yamada M, Sato T, Shimohata T, Hayashi S, Igarashi S, Tsuji S, Takahashi H: Interaction between neuronal intranuclear inclusions and promyelocytic leukemia protein nuclear and coiled bodies in CAG repeat diseases. Am J Pathol 159: 1785-1795, 2001 Takahashi H, Egawa H, Piao Y-S, Hayashi S, Yamada M, Shimohata T, Oyanagi K, Tsuji S: Brain Res 919: 12-19, 2001 Wakabayashi K, Engelender S, Tanaka Y, Yoshimoto M, Mori F, Tsuji S, Ross CA, Takahashi H: Immunocytochemical localization of synphilin-1, an a-synuclein-associated protein, in neurodegenerative disorders. Acta Neuropathol 103: 209-214, 2001 Nozaki K, Onodera O, Takano H, Tsuji S: Amino acid sequences flanking polyglutamine stretches influence their potential for aggregate formation. NeuroReport 12: 3357-3364, 2001 Asaka T, Ikeuchi K, Okino S, Takizawa Y, Satake R, Nitta E, Komai K, Endo K, Higuchi S, Oyake T, Yoshimura T, Suenaga A, Uyam E, Saito T, Konagaya M, Sunohara N, Namba R, Takada H, Honke K, Nishina M, Tanaka H, Shinagawa M, Tanaka K, Matsushima A, Tsuji S, Takamori M: Homozygosity and linkage disequilibrium mapping of autosomal recessive distal myopathy (Nonaka distal myopathy). J Hum Genet 46: 649-655, 2001 Nucifora FC Jr, Sasaki M, Peters MF, Huang H, Cooper JK, Yamada, M, Takahashi H, Tsuji S, Troncoso J, Dawson VL, Dawson, TM, Ross CA: Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science 291: 2423-2428, 2001 Funayama M, Hasegawa K, Kowa H, Saito M, Tsuji S, Obata F: A new locus for Parkinson’s disease (PARK8) maps to chromosome 12p11.2-q13.1. Ann Neurol 51: 296-301, 2002 Takahashi T, Igarashi S, Kimura T, Hozumi I, Kawachi I, Onodera O, Takano H, Saito M, Tsuji S: Japanese cases of familial hemiplegic migraine with cerebellar ataxia carrying a T666M mutation in the CACNA1A gene. J Neurol Neurosurg Psychiat 72: 675-680, 2002 Toyoshima I, Sugawara M, Kato K, Wada C, Shimohata T, Koide R, Onodera O, Tsuji S: Time course of polyglutamine aggregate body formation and cell death: Enhanced growth in nucleus and an interval for cell death. J Neurosci Res 68: 442-448, 2002 Shimohata T, Sato A, Burke JR, Strittmatter WJ, Tsuji S, Onodera O: Expanded polyglutamine stretchers form an ‘aggresome’. Neurosci Lett 323: 215-218, 2002 Shimazaki H, Takiyama Y, Sakoe K, Ikeguchi K, Niijima K, Kaneko J, Namekawa M, Ogawa T, Date H, Tsuji S, Nakano I, Nishizawa M: Early-onset ataxia with ocular motor apraxia and hypoalbuminemia. Neurology 59: 590-595, 2002 Arai A, Tanaka K, Igarashi S, Kobayashi H, Asaka T, Date H, Saito M, Tanaka H, Kawasaki S, Uyama E, Mizusawa H, Fukuhara N, Tsuji S: A novel mutation in the GNE gene and a linkage disequilibrium in Japanese pedigrees. Ann Neurol 52: 516-519, 2002 Silveria I, Miranda C, Guimarães L, Moreira M-C, Alonso I, Mendonça P, Ferro A, Pinto-Basto J, Coelho J, Ferreirinha F, Poirier J, Parreira E, Vale J, Januário C, Barbot C, Tuna A, Barros J, Koide R, Tsuji S, Holmes SE, Margolis RL, Jardim L, Pandolfo M, Coutinho P, Sequeiros J: Trinucleotide repeats in 202 families with ataxia. A small expanded (CAG)n allele at the SCA17 locus. Arch Neurol 59: 623-629, 2002 Ikeuchi T, Nomura Y, Segawa M, Ozelius LJ, Shimohata T, Tsuji S: Multiple founder effects in Japanese families with primary torsion dystonia harboring the GAG deletion in the Tor1A (DYT1) gene. Neurogenetics 4: 105-106, 2002 Wakabayashi K, Engelender S, Tanaka Y, Yoshimoto M, Mori F, Tsuji S, Ross CA, Takahashi H: Immunocytochemical localization of synphilin-1, an α-synuclein-associated protein, in neurodegenerative disorders. Acta Neuropathol 103: 209-214, 2002 Yamada M, Sato T, Tsuji S, Takahashi H: Oligodendrocytic polyglutamine pathology in dentatorubral-pallidoluysian atrophy. Ann Neurol 52: 670-674, 2002 Yamada M, Tsuji S, Takahashi H: Involvement of lysosomes in the pathogenesis of CAG repeat diseases. Ann Neurol 52: 498-503, 2002 Yamada M, Tsuji S, Takahashi H: Genotype-phenotype correlation in CAG-repeat diseases. Neuropathology 22: 317-322, 2002 Iwata A, Maruyama M, Akagi T, Hashikawa T, Kanazawa I, Tsuji S, Nukina N: Alpha-synuclein degradation by serine protease neurosin: Implication for pathogenesis of synucleinopathies. Hum Mol Genet 12: 2625-2635, 2003 Sanpei K, Ikeuchi T, Tsuji S: DIRECT technologies for molecular cloning of genes containing expanded CAG repeats. Methods Mol Biol 217: 73-81, 2003 Sekijima Y, Hashimoto T, Onodera O, Date H, Okano T, Naito K, Tsuji S, Ikeda S: Severe generalized dystonia as a presentation of a patient with aprataxin gene mutation. Mov Disord 18: 1198-1200, 2003 Yabe I, Sasaki H, Chen DH, Raskind WH, Bird TD, Yamashita I, Tsuji S, Kikuchi S, Tashiro K: Spinocerebellar ataxia type 14 caused by a mutation in protein kinase C gamma. Arch Neurol 60: 1749-1751, 2003 Shimohata T, Kimura T, Nishizawa M, Onodera O, Tsuji S: Five year follow up of a patient with spinal and bulbar muscular atrophy treated with leuprorelin. J Neurol Neurosurg Psychiat 75: 1206-207, 2004 Sano Y, Date H, Igarashi S, Onodera O, Oyake M, Takahashi T, Hayashi S, Morimatsu M, Takahashi H, Makifuchi T, Fukuhara N, Tsuji S: Aprataxin, the causative protein for early-onset ataxia with ocular motor apraxia and hypoalbuminemia, is a nuclear protein with a potential role as a nucleotide repair protein. Ann Neurol 55: 241-249, 2004 Züchner S, Mersiyanova IV, Muglia M, Bissar-Tadmouri N, Rochelle J, Dadali EL, Zappia M, Nelis E, Patitucci A, Senderek J,. Parman Y,. Evgrafov O, Jonghe PD, Takahashi Y, Tsuji S, Pericak-Vance MA, Quattrone A, Battaloglu E, Polyakov AV, Timmerman V, Schröder JM, Vance JM, Battologlu E: Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A. Nat Genet 36: 449-451, 2004 Yamada M, Tan S-H, Inenaga C, Tsuji S, Takahashi H: Sharing of polyglutamine transport by the neuronal nucleus and cytoplasm in CAG-repeat diseases. Neuropathol Appl Neurobiol 30: 665-675, 2004 Date H, Igarashi S, Sano Y, Takahashi T, Takahashi T, Takano H, Tsuji S, Nishizawa S, Onodera O: The FHA domain of aprataxin (APTX) interacts with the C terminal region of XRCC1. Biochem Biophys Res Commun 325: 1279-1285, 2004 Shimohata M, Shimohata T, Igarashi S, Naruse S, Tsuji S: Interference of CREB-dependent transcriptional activation by expanded polyglutamine stretches - Augmentation of transcriptional activation as a potential therapeutic strategy for polyglutamine diseases. J Neurochem 93: 654-663, 2005 岩坪　威　(東京大学・大学院薬学系研究科) Okochi M, Walter J, Koyama A, Nakajo S, Baba M, Iwatsubo T, Meijer L, Kahle P, Haass C: Constitutive phosphorylation of the Parkinson’s disease associated α-synuclein. J Biol Chem 275: 390-397, 2000 Wakabayashi K, Fukushima T, Koide R, Horikawa Y, Hasegawa M, Watanabe Y, Noda T, Eguchi I, Morita T, Yoshimoto M, Iwatsubo T, Takahashi H: Juvenile-onset generalized neuroaxonal dystrophy (Hallervorden-Spatz disese) with diffuse neurofibrillary and Lewy body pathology. Acta Neuropathol 99: 331-336, 2000 Maruyama K, Usami M, Kametani F, Tomita T, Iwatsubo T, Saido TC, Mori H, Ishiura S: Molecular interactions between presenilin and calpain: Inhibition of m-calpain protease activity by presenilin-1, 2 and cleavage of presenilin-1 by m-calpain. Int J Mol Med 5: 269-273, 2000 Nishizawa T, Nagao T, Iwatsubo T, Forte JG, Urushidani T: Molecular cloning and characterization of a novel CLIC-related protein, parchorin, expressed in water-secreting cells. J Biol Chem 275: 11164-11173, 2000 Yasuda M, Takamatsu J, D’Souza I, Crowther RA, Kawamata T, Hasegawa M, Hasegawa H, Spillantini MG, Tanimukai S, Poorkaj P, Varani L, Iwatsubo T, Goedert M, Schellenberg G, Tanaka C: A novel mutation at position +12 in the intron following exon 10 of the tau gene in familial frontotemporal dementia (FTD-Kumamoto). Ann Neurol 47: 422-429, 2000 Arima K, Kowalska A, Hasegawa M, Mukoyama M, Watanabe R, Kawai M, Takahashi K, Iwatsubo T, Tabira T, Sunohara N: Two brothers of frontotemporal dementia and parkinsonism with an N279K mutation of tau gene. Neurology 54: 1787-1795, 2000 Saura CA, Tomita T, Soriano S, Takahashi M, Leem J-Y, Honda T, Koo EH, Iwatsubo T, Thinakaran G: The non-conserved hydrophilic loop domain of presenilin is neither required for presenilin endoproteolysis nor enhanced Aβ42 production mediated by FAD-linked PS variants. J Biol Chem275: 17136-17142, 2000 Yamagata K, Urakami K, Wakutani Y, Adachi Y, Iwatsubo T, Mann DMA, Sato K, Nakashima K: Increased levels of apolipoprotein E mRNA in the brains of patients with Down’s syndrome. Alz Rep 3: 77-82, 2000 Takeuchi A, Irizarry MC, Duff K, Saido TC, Hsiao Ashe K, Hasegawa M,Mann DMA, Hyman BT, Iwatsubo T: Age-related amyloid b deposition in transgenic mice overexpressing both presenilin 1 and amyloid β precursor protein Swedish mutant is not associated with global neuronal loss. Am J Pathol157: 331-339, 2000 Yamazaki M, Arai Y, Baba M, Iwatsubo T, Mori O, Katayama Y, Oyanagi K: α-Synuclein inclusions in amygdala in the brains of patients with Parkinsonism-Dementia Complex of Guam. J Neuropathol Exp Neurol 59: 585-591, 2000 Wakabayashi K, Shibasaki Y, Hasegawa M, Horikawa Y, Soma Y, Hayashi S, Morit T, Iwatsubo T, Takahashi H: Primary progressive aphasia with focal glial tauopathy. Neuropathol Appl Neurobiol 26: 477-481, 2000 Lambert JC, Mann D, Goumidi L, Harris J, Pasquier F, Frigard B, Cottel D, Lendon C, Iwatsubo T, Amouyel P, Chartier-Harlin MC: A FE65 polymorphism associated with risk of developing sporadic late-onset Alzheimer's disease but not with Aβ loading in brains. Neurosci Lett 293: 29-32, 2000 Takahashi M, Dore S, Ferris CD, Tomita T, Sawa A, Wolosker H, Borchelt DR, Iwatsubo T, Kim S-H, Thinakaran G, Sisodia SS, Snyder SH: Amyloid precursor proteins inhibit heme oxygenase activity and augument neurotoxicity in Alzheimer’s disease. Neuron 28: 461-473, 2000 Pickering-Brown S, Baker M, Yen S-H, Liu W-K, Hasegawa M, Cairns N, Lantos PL, Rossor M, Iwatsubo T, Davies Y, Allsop D, Furlong R, Owen F, Hardy J, Mann D, Hutton M: Pick’s disease is associated with mutations in the tau gene. Ann Neurol 48: 859-867, 2000 Taniguchi S, Fujita Y, Hayashi S, Kakita A, Takahashi H, Murayama S, Saido TC, Hisanaga S, Iwatsubo T, Hasegawa M: Calpain-mediated degradation of p35 to p25 in postmortem human and rat brains. FEBS Lett 489: 46-50, 2001 Lee J-A, Long Z, Nimura N, Iwatsubo T, Imai K, Homma H: Localization, transport, and uptake of D-aspartate in the rat adrenal and pituitary glands. Arch Biochem Biophys 385: 242-249, 2001 Lambert J-C, Mann D, Goumidi L, Harris J, Amouyel P, Iwatsubo T, Lendon CL, Chartier-Harlin M-C: Effect of the APOE promoter polymorphisms on cerebral amyloid peptide deposition in Alzheimer’s disease. Lancet 357: 608-609, 2001 Ohya S, Morohashi Y, Muraki K, Tomita T, Watanabe M, Iwatsubo T, Imaizumi Y: Molecular cloning and expression of the novel splice variants of K+ channel-interacting protein 2 (KChIP2). Biochem Biophys Res Commun 282: 96-102, 2001 Piao Y-S, Hayashi S, Hasegawa M, Wakabayashi K, Yamada M, Yoshimoto M, Ishikawa A, Iwatsubo T, Takahashi T: Co-localization of a-synuclein and phosphorylated tau in neuronal and glial cytoplasmic inclusions in a patient with multiple system atrophy of long duration. Acta Neuropathol 101: 285-293, 2001 Kuzuhara S, Kokubo Y, Sasaki R, Narita Y, Yabana T, Hasegawa M, Iwatsubo T: Familial amyotrophic lateral sclerosis and parkinsonism-dementia complex of the Kii peninsula of Japan: clinical and neuropathological study and tau analysis. Ann Neurol 49: 501-511, 2001 Mann DMA, McDonagh AM, Pickering-Brown SM, Kowa H, Iwatsubo T: Amyloid β protein deposition in patients with frontotemporal lobar degeneration: relationship to age and apolipoprotein E genotype. Neurosci Lett 304: 161-164, 2001 Verkkoniemi A, Kalimo H, Paetau A, Somer M, Iwatsubo T, Hardy J, Haltia M: Variant Alzheimer’s disease with spastic paraparesis: Neuropathological phenotype. J Neuropathol Exp Neurol 60: 483-492, 2001 Mann DMA, Pickering-Brown SM, Takeuchi A,Iwatsubo T:Amyloid angiopathy and variability in amyloid β deposition is determined by mutation position in presenilin-1 linked Alzheimer’s disease. Am J Pathol 158: 2165-2175, 2001 Shirotani K, Tsubuki S, Iwata N, Takaki Y, Harigaya W, Maruyama K, Kiryu-Seo S, Kiyama H, Iwata H, Tomita T, Iwatsubo T, Saido TC: Neprilysin degrades both amyloid β peptide 1-40 and 1-42 most effectively among thiorphan- and phosphoramidon-sensitive endopeptidases. J Biol Chem 276: 21895-21901, 2001 Iwata H, Tomita T, Maruyama K, Iwatsubo T: Subcellular compartment and molecular subdomain of β-amyloid precursor protein relevant to the Aβ42-promoting effects of Alzheimer mutant presenilin 2. J Biol Chem 276: 21678-21685, 2001 Lambert J-C, Mann D, Harris JM, Chartier-Harlin M-C, Cummings A, Coates J, Lemmon H, St-Clair D, Iwatsubo T, Lendon C: The -48 CT polymorphism in the presenilin 1 promoter is associated with an increased risk of developing Alzheimer’s disease and an increased Aβ load in brain. J Med Genet 38: 353-355, 2001 Mann DMA, Takeuchi A, Sato S, Cairns NJ, Lantos PL, Rossor MN, Haltia M, Kalimo H, Iwatsubo T: Cases of Alzheimer’s disease due to deletion of exon 9 of the presenilin-1 gene show an unusual but characteristic β amyloid pathology known as “cotton wool” plaques. Neuropathol Applied Neurobiol 27: 189-196, 2001 Mori H, Motoi Y, Kobayashi T, Hasegawa M, Yamamura A, Iwatsubo T, Mizuno Y: Tau accumulation in a patient with pallidonigroluysian atrophy. Neurosci Lett 309: 89-92, 2001 Tomita T, Watabiki T, Takikawa R, Morohashi Y, Takasugi N, Kopan R, De Strooper B, Iwatsubo T: The first proline of PALP motif at the C terminus of presenilins is obligatory for stabilization, complex formation and γ-secretase activities of presenilins. J Biol Chem 276: 33273-33281, 2001 Kahle PJ, Neumann M, OzmenL, MüllerV, Odoy S, JacobsenH, IwatsuboT, TrojanowskiJQ, TakahashiH, Wakabayashi K, BogdanovicN, RiedererP, KretzschmarHA, Haass C: Selective insolubility of α-synuclein in human Lewy body diseases is recapitulated in a transgenic mouse model. Am J Pathol 159: 2215-2225, 2001 Ikegaya YM, Kim J-A, Baba M, Iwatsubo T, Nishiyama N, Matsuki N: Rapid and reversible changes in dendrite morphology and synaptic efficacy following NMDA receptor activation: Implication for a cellular defense against excitotoxicity. J Cell Sci 114: 4083-4093, 2001 Fujiwara H, Hasegawa M, Dohmae N, Kawashima A, Masliah E, Goldberg MS, Shen J, Takio K, Iwatsubo T: α-Synuclein is phosphorylated in synucleinopathy lesions. Nat Cell Biol 4: 160-164, 2002 Hashimoto T, Wakabayashi T, Watanabe A, Kowa H, Hosoda R, Nakamura A, Kanazawa I, Arai T, Takio K, Mann DMA, Iwatsubo T: CLAC: a novel Alzheimer amyloid plaque component derived from a transmembrane precursor, CLAC-P/collagen type XXV. EMBO J 21: 1524-1534, 2002 Hayashi S, Toyoshima Y, Hasegawa M, Umeda Y, Wakabayashi K, Tokiguchi S, Iwatsubo T, Takahashi H: Late-onset frontotemporal dementia with a novel exon 1 (Arg5His) tau gene mutation. Ann Neurol 51: 525-530, 2002 Lambert J-C, Thaker U, Green EK, Harris JM, McDonagh AM, Iwatsubo T, Chartier-Harlin MC, Pickering-Brown SM, Lendon CL, Mann DMA: The -48 C/T polymorphism within the promotor region of the presenilin-1 gene does not influence the extent of tau pathology within the brain in sporadic Alzheimer’s disease. Alz Rep 5: 13-16, 2002 Morohashi Y, Hatano N, Ohya S, Takikawa R, Watabiki T, Takasugi N, Imaizumi Y, Tomita T, Iwatsubo T: Molecular cloning and characterization of CALP/KChIP4, a novel EF-hand protein interacting with presenilin 2 and voltage-gated potassium channel subunit Kv4. J Biol Chem 277: 14965-14975, 2002 Miake H, Mizusawa H, Iwatsubo T, Hasegawa M: Biochemical characterization of the core structure of α-synuclein filaments. J Biol Chem 277: 19213-19219, 2002 Green EK, Thaker U, McDonagh AM, Iwatsubo T, Lambert JC, Chartier-Harlin MC, Harris JM, Pickering-Brown SM, Lendon CL, Mann DMA: A polymorphism within intron 11 of the tau gene is not increased in frequency in patients with sporadic Alzheimer's disease, nor does it influence the extent of tau pathology in the brain. Neurosci Lett 324: 113-116, 2002 Tomita T, Katayama R, Takikawa R, Iwatsubo T: Complex N-glycosylated form of nicastrin is stabilized and selectively bound to presenilin fragments. FEBS Lett 520: 117-121, 2002 Green EK, Harris JM, Lemmon H, Lambert JC, Chartier-Harlin MC, St Clair D, Mann DMA, Iwatsubo T, Lendon CL:Are interleukin-1 gene polymorphisms risk factors or disease modifiers in Alzheimer’s disease. Neurology 58: 1566-1568, 2002 Kahle PJ, Neumann M, Ozmen L, Muller V, Jacobsen H, Spooren W, Fuss B, Mallon B, Macklin WB, Fujiwara H, Hasegawa M, Iwatsubo T, Kretzschmar HA, Haass C: Hyperphosphorylation and insolubility of α-synuclein in transgenic mouse oligodendrocytes. EMBO Rep 3: 583-588, 2002 Hazeki N, Tsukamoto T, Yazawa I, Koyama M, Hattori S, Someki I, Iwatsubo T, Nakamura K, Goto J, Kanazawa I: Ultrastructure of nuclear aggregates formed by expressing an expanded polyglutamine. Biochem Biophys Res Commun 294: 429-440, 2002 Araria-Goumidi L, Lambert JC, Mann DMA, Lendon C, Frigard B, Iwatsubo T, Cottel D, Amouyel P, Chartier-Harlin MC: Association study of three polymorphisms of TGF-β1 gene with Alzheimer’s disease. J Neurol Neurosurg Psychiat 73: 62-64, 2002 Lendon CL, Thaker U, Harris JM, McDonagh AM, Lambert JC, Chartier-Harlin MC, Iwatsubo T, Pickering-Brown SM, Mann DMA: The angiotensin 1-converting enzyme insertion (I)/deletion (D) polymorphism does not influence the extent of amyloid or tau pathology in patients with sporadic Alzheimer’s disease. Neurosci Lett 328: 314-318, 2002 Ohara S, Tsuyuzaki J, Oide T, Arai H, Higuchi S, Hasegawa M, Iwatsubo T: A clinical and neuropathological study of an unusual case of sporadic tauopathy. A variant of corticobasal degeneration? Neurosci Lett 330: 84-88, 2002 OkochiM, SteinerH, Fukumori A, Tanii H, Tomita T, Tanaka T, Iwatsubo T, Kudo T, Haass C, Takeda M: Presenilins mediate a dual intramembranous γ-secretase cleavage of Notch-1. EMBO J 21: 5408-5416, 2002 Watanabe T, Nakagawa K, Ohata S, Kitagawa D, Nishitai G, Seo J, Tanemura S, Shimizu N, Kishimoto H, Wada T, Aoki J, Arai H, Iwatsubo T, Mochita M, Watanabe T, Satake M, Ito Y, Matsuyama T, Mak TW, Penninger JM, Nishina H, Katada T: SEK1/MKK4-mediated SAPK/JNK signaling participates in embryonic hepatoblast proliferation via a pathway different from NF-kB-induced anti-apoptosis. Dev Biol250: 332-347, 2002 Hayes A, Thakera U, Iwatsubo T, Pickering-Brown SM, Mann DMA: Pathological relationships between microglial cell activity and tau and amyloid β protein in patients with Alzheimer’s disease.Neurosci Lett 331: 171-174, 2002 Matsushita S, Arai H, Okamura N, Ohmori T, Takasugi Matsui T, Murayama M, Iwatsubo T, Higuchi S: Clinical and biomarker investigation of a patient with a novel PS-1 mutation (A431V) in the mild cognitive impairment stage of Alzheimer’s disease. Biol Psychiat 52: 907-910, 2002 Neumann M, Kahle PJ, Giasson BI, Ozmen L, Borroni E, Spooren W, Muller V, Odoy S, Fujiwara H, Hasegawa M, Iwatsubo T, Trojanowski JQ, Kretzschmar HA, Haass C: Misfolded proteinase K-resistant and hyperphosphorylated α-synuclein in aged transgenic mice and in Lewy body disease patients. J Clin Invest 110: 1429-1439, 2002 Leem JY, Saura CA, Pietrzik C, Kim S-H, Veselits ML, Christianson J, Wanamaker C, Tomita T, Gasparini L, Iwatsubo T, Xu H, Green WN, Koo E-H, Thinakaran G: A role for presenilin 1 in regulating the delivery of amyloid precursor protein to the cell surface. Neurobiol Dis 11: 64-82, 2002 Hasegawa M, Fujiwara H, Nonaka T, Wakabayashi K, Takahashi H, Lee VM-Y, Trojanowski JQ, Mann DMA, Iwatsubo T: Phosphorylated α-synuclein is ubiquitinated in α-synucleinopathy lesions. J Biol Chem 277: 49071-49076, 2002 Takasugi N, Takahashi Y, Morohashi Y, Tomita T, Iwatsubo T: The mechanism of γ-secretase activities through high molecular weight complex formation of presenilins is conserved in Drosophila melanogaster and mammals. J Biol Chem 277: 50198-50205, 2002 Takahashi M, Kanuka H, Fujiwara H, Koyama A, Hasegawa M, Miura M, Iwatsubo T: Phosphorylation of α-synuclein characteristic of synucleinopathy lesions is recapitulated in α-synuclein transgenic Drosophila. Neurosci Lett 336: 155-158, 2003 Thaker U, McDonagh AM, Iwatsubo T, Lendon CL, Pickering-Brown SM, Mann DMA: Tau load is associated with apolipoprotein E genotype and the amount of amyloid β protein Aβ40 in sporadic and familial Alzheimer’s disease. Neuropathol Appl Neurobiol 29: 35-44, 2003 Murakami D, Okamoto I, Nagano O, Kawano Y, Tomita T, Iwatsubo T, De Strooper B, Yumoto E, Saya H: Presenilin-dependent γ-secretase activity mediates the intramembranous cleavage of CD44.Oncogene 22: 1511-1516, 2003 Takasugi N, Tomita T, Hayashi I, Tsuruoka M, Niimura M, Takahashi Y, Thinakaran G, Iwatsubo T: The role of presenilin cofactors in the γ-secretase complex. Nature 422: 438-441, 2003 Kamenetz F, Tomita T, Hsieh H, Seabrook G, Borchelt D, Iwatsubo T, Sisodia S, Malinow R: APP processing and synaptic function. Neuron 37: 925-937, 2003 Takahashi Y, Hayashi I, Tominari Y, Rikimaru K, Morohashi Y, Kan T, Natsugari H, Fukuyama T, Tomita T, Iwatsubo T: Sulindac sulfide is a non-competitive γ-secretase inhibitor that preferentially reduces Aβ42 generation. J Biol Chem 278: 18664-18670, 2003 Saito Y, Kawashima A, Ruberu NN, Fujiwara H, Koyama S, Sawabe M, Arai T, Nagura H, Yamanouchi H, Hasegawa M, Iwatsubo T, Murayama S: Accumulation of phosphorylated α-synuclein in aging human brain. J Neuropathol Exp Neurol 62: 644-654, 2003 Mori F, Piao Y-S, Hayashi S, Fujiwara H, Hasegawa M, Yoshimoto M, Iwatsubo T, Takahashi H, Wakabayashi K: α-synuclein accumulates in purkinje cells in Lewy body disease but not in multiple system atrophy. J Neuropathol Exp Neurol 62: 812-819, 2003 Abe Y, Kouyama K, Tomita T, Tomita Y, Ban N, Nawa M, Matsuoka M, Niikura T, Aiso S, Kita Y, Iwatsubo T, Nishimoto I: Analysis of neurons created from wild-type and Alzheimer’s mutation knock-in embryonic stem cells by a highly efficient differentiation protocol. J Neurosci 23: 8513-8525, 2003 Kan T, Tominari Y, Morohashi Y, Natsugari H, Tomita T, Iwatsubo T, Fukuyama T: Solid-phase synthesis of photoaffinity probes: highly efficient incorporation of biotin-tag and cross-linking groups. Chem Commun 17: 2244-2245, 2003 Schroeter EH, Ilagan MXG, Brunkan AL, Hecimovic S, Li Y-M, Xu M, Lewis HD, Saxena MT, De Strooper B, Coonrod A, Tomita T, Iwatsubo T, Moore CL, Shearman M, Goate A, Wolfe MS, Kopan R: A presenilin dimer at the core of the γ-secretase enzyme? Insights from parallel analysis of Notch 1 and APP proteolysis.Proc Natl Acad Sci USA 100: 13075-13080, 2003 Soderberg L, Zhukareva V, Bogdanovic N, Hashimoto T, Winblad B, Iwatsubo T, Lee VM-Y, Trojanowski JQ, Naslund J: Molecular identification of AMY, and Alzheimer disease amyloid-associated protein. J Neuropathol Exp Neurol 62: 1108-1117, 2003 Taniguchi S, McDonaghAM, Pickering-Brown SA, Umeda Y, Iwatsubo T, Hasegawa M, Mann DMA: The neuropathology of frontotemporal lobar degeneration with respect to the cytological and biochemical characteristics of tau protein. Neuropathol Appl Neurol 30: 1-18, 2004 Matsubayashi H, Sese S, Lee J-S, Shirakawa T, Iwatsubo T, Tomita T, Yanagawa S-i: Biochemical characterization of Drosophila Wingless signaling pathway based on RNA interference. MolCell Biol 24: 2012-2024, 2004 Lambert JC, Mann D, Harris J, Araria-Goumidi L, Chartier-Harlin MC, Cottel D, Iwatsubo T, Amouyel P, Lendon C: Association study of Notch 4 polymorphisms with Alzheimer’s disease. J Neurol Neurosurg Psychiat 75: 377-381, 2004 Tanaka M, Kim YM, Lee G, Junn E, Iwatsubo T, Mouradian M: Aggresomes formed by α-synuclein and synphilin-1 are cytoprotective. J Biol Chem 279: 4625-4631, 2004 Fuwa H, Okamura Y, Morohashi Y, Tomita T, Iwatsubo T, Kan T, Fukuyama T, Natsugari H: Highly efficient synthesis of medium-sized lactams via intramolecular Staudinger-aza-Wittig reaction of w-azido pentafluorophenyl ester: synthesis and biological evaluation of LY411575 analogues. Tetrahedron Lett 45: 2323-2396, 2004 Nishie M, Mori F, Fujiwara H, Hasegawa M, Yoshimoto M, Iwatsubo T, Takahashi H, Wakabayashi K: Accumulation of phosphorylated α-synuclein in the brain and peripheral ganglia of patients with multiple system atrophy. Acta Neuropathol 107: 292-298, 2004 Umeda Y, Taniguchi S, Arima K, Piao Y-SH, Takahashi H, Iwatsubo T, Mann D, Hasegawa M: Alterations in human tau transcripts correlate with those of neurofilament in sporadic tauopathies. Neurosci Lett 359: 151-154, 2004 Kan T, Tominari Y, Rikimaru K, Morohashi Y, Natsugari H, Tomita T, Iwatsubo T, Fukuyama T: Parallel synthesis of DAPT derivatives and their γ-secretase-inhibitory activity. Bioorg Med Chem Lett 14: 1983-1985, 2004 Busby V, Goossens S, Nowotny P, Hamilton G, Smemo S, Harold D, Turic D, Jehu L, Myers A, Womick M, Woo D, Compton D, Doil LM, Tacey KM, Lau KF, Al-Saraj S, Killick R, Pickering-Brown S, Moore P, Hollingworth P, Archer N, Foy C, Walter S, Lendon C, Iwatsubo T, Morris JC, Norton J, Mann D, Janssens B, Hardy J, O’Donovan M, Jones L, Williams J, Holmans P, Owen MJ, Grupe A, Powell J, van Hengel J, Goate A, Van Roy F, Lovestone S: α-T-catenin is expressed in human brain and interacts with the Wnt signalling pathway but is not responsible for linkage to chromosome 10 in Alzheimer’s disease. Neuromolecular Med 5: 133-146, 2004 Kawasumi M, Chiba T, Yamada M, Miyamae-Kaneko M, Matsuoka M, Nakahara J, Tomita T, Iwatsubo T, Kato S, Aiso S, Nishimoto I, Kouyama K: Targeted introduction of V642I mutation in amyloid precursor protein gene causes functional abnormality resembling early stage of Alzheimer’s disease in aged mice. Eur J Neurosci 19: 2826-2838, 2004 Wakutani Y, Watanabe K, Adachi Y, Wada-Isoe K, Urakami K, Ninomiya H, Saido TC, Hashimoto T, Iwatsubo T, Nakashima K: Novel amyloid precursor protein missense mutation (D678N) in familial Alzheimer’s disease. J Neurol Neurosurg Psychiat 75: 1039-1042, 2004 Zhang Y, Hayes A, Pritchard A, Haque MS, Lemmon H, Harris J, Cumming A, Lambert JC, Chartier-Harlin MC, St Clair D, Iwatsubo T, Mann DM, Lendon CL: Interleukin-6 promoter polymorphism: risk and pathology of Alzheimer’s disease. Neurosci Lett 362: 99-102, 2004 Kowa H, Sakakura T, Matsuura Y, Wakabayashi T, Mann DMA, Duf K, Tsuji S, Hashimoto T, Iwatsubo T: Mostly separate distributions of CLAC- versus Aβ40- or thioflavin S-reactivities in senile plaques reveal two distinct subpopulations of β-amyloid deposits. Am J Pathol 165: 273-281, 2004 Nagano O, Murakami D, Hartmann D, De Strooper B, Saftig P, Iwatsubo T, Nakajima M, Shinohara M, Saya H: Cell-matrix interaction via CD44 is independently regulated by different metalloproteinases activated in response to extracellular Ca2+ influx and PKC activation. J Cell Biol 165: 893-902, 2004 Hirai Y, Fujita SC, Iwatsubo T, Hasegawa M: Phosphorylated α-synuclein in normal mouse brain. FEBS Lett 572: 227-232, 2004 Hayashi I, Urano Y, Fukuda R, Isoo N, Kodama T, Hamakubo T, Tomita T, Iwatsubo T: Selective reconstitution and recovery of functional γ-secretase complex on budded baculovirus particles. J Biol Chem 279: 38040-38046, 2004 Yamada M, Iwatsubo T, Mizuno Y, Mochizuki H: Overexpression of α-synuclein in rat substantia nigra results in loss of dopaminergic neurons, phosphorylation of α-synuclein and activation of caspase-9. Resemblance to pathogenetic changes in Parkinson’s disease. J Neurochem 91: 451-461, 2004 Lo Bianco C, Schneider BL, Bauer M, Sajadi A, Brice A, Iwatsubo T, Aebischer P: Lentiviral vector delivery of parkin prevents dopaminergic degeneration in an α-synuclein rat model of Parkinson’s disease. Proc Natl Acad Sci USA 101: 17510-17515, 2004 Nonaka T, Iwatsubo T, Hasegawa M: Ubiquitination of α-synuclein. Biochemistry 44: 361-368, 2005 Taniguchi S, Suzuki N, Masuda M, Hisanaga S, Iwatsubo T, Goedert M, Hasegawa M: Inhibition of heparin-induced tau filament formation by phenothiazines, polyphenols and porphyrins. J Biol Chem 280: 7614-7623, 2005 Osada Y, Hashimoto T, Nishimura A, Matsuo Y, Wakabayashi T, Iwatsubo T: CLAC binds to amyloid β peptides through the positively-charged amino acid cluster within the collagenous domain 1 and inhibits formation of amyloid fibrils. J Biol Chem 280: 8596-8605, 2005 Niimura M, Isoo N, Takasugi N, Tsuruoka M, Tei-Ui K, Saigo K, Morohashi Y, Tomita T, Iwatsubo T: Aph-1 contributes to the stabilization and trafficking of the γ-secretase complex through mechanisms involving inter- and intramolecular interactions. J Biol Chem 280: 12967-12975, 2005 Urano Y, Hayashi I, Isoo N, Reid PC, Shibasaki Y, Noguchi N, Tomita T, Iwatsubo T, Hamakubo T, Kodama T: Association of active γ-secretase complex with lipid rafts. J Lipid Res 46: 904-912, 2005 Iseki E, Marui W, Yamamoto R, Togo T, Katsuse O, Kato M, Iwatsubo T, Kosaka K, Arai H: The nigro-striatal and nigro-amygdaloid pathways undergo different degeneration processes in brains of dementia with Lewy bodies. Neurosci Lett 380: 161-165, 2005 Machida Y, Chiba T, Takayanagi A, Tanaka Y, Asanuma M, Ogawa N, Koyama A, Iwatsubo T, Ito S, Jansen PH, Shimizu N, Tanaka K, Mizuno Y, Hattori N: Common anti-apoptotic roles of parkin and α-synuclein in human dopaminergic cells. Biochem Biophys Res Commun 332: 233-240, 2005 Pritchard A, Harris J, Pritchard CW, St Clair D, Lemmon H, Lambert JC, Chartier-Harlin MC, Hayes A, Thaker U, Iwatsubo T, Mann DM, Lendon C: Association study and meta-analysis of low-density lipoprotein receptor related protein in Alzheimer’s disease. Neurosci Lett 382: 221-226, 2005 Smith WW, Margolis RL, Li X, Troncoso JC, Lee MK, Dawson VL, Dawson TM, Iwatsubo T, Ross CA: α-Synuclein phosphorylation enhances Lewy body-like inclusion formation in SH-SY5Y cells. J Neurosci 25: 5544-5552, 2005 祖父江　元　(名古屋大学・大学院医学研究科) Mizuno K, Wakai M, Takeda A, Sobue G: Medial temporal atrophy and memory impairment in early stage of Alzhermer’s disease: an MRI volumetric and memory assessment study. J Neurol Sci 173: 18-24, 2000 Takeuchi H, Misu K, Hattori N, Nagamatsu M, Sobue G: Immunoglobulin therapy for idiopathic chronic sensory ataxic neuropathy. Neurology 54: 1008-1010, 2000 Kobayashi Y, Kume A, Li M, Doyu M, HataM, Ohtsuka K, Sobue G: Chaperones, Hsp70 and Hsp40, suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract. J Biol Chem 275: 8772-8778, 2000 Inukai A, Kuru S, Liang Y, Takano A, Kobayashi Y, Sakai M, Doyu M, Sobue G: Expression of HLA-DR and its enhancing molecules in muscle fibers in polymyositis. Muscle Nerve 23: 385-392, 2000 Mutoh T, Sobue G, Hamano T, Kuriyama M, Hirayama M, Yamamoto M, Mitsuma T: Decreased phosphorylation levels of TrkB neurotrophin receptor in the spinal cords from patients with amyotrophic lateral sclerosis. Neurochem Res 25: 239-245, 2000 Kuru S, Inukai A, Liang Y, Doyu M, Takano A, Sobue G: Tumor necrosis factor-α expression in muscles of polymyositis and dermatomositis. Acta Neuropathol 99: 585-588, 2000 Takano A, Bönneman CG, Honda H, Sakai M, Feener CA, Kunkel LM, Sobue G: Intrafamilial phenotype variation in limb-girdle muscular dystrophy type 2c with compound heterozygous mutations. Muscle Nerve 23: 807-810, 2000 Terao S, Takeda A, Miura N, Izumi M, Ito E., Mitsuma T, Sobue G: Clinical and pathophysiological features of amaurosis fugax in Japanese stroke patients. Intern Med 39: 118-122, 2000 Yoshihara T, Yamamoto M, Doyu M, Misu K, Hattori N, Hasegawa Y, Mokuno K, Mitsuma T, Sobue G: Mutations in the peripheral myelin protein zero and connexin 32 genes detected by non-isotopic RNase cleavage assay and their phenotype in Japanese patients with Charcot-Marie-Tooth disease. Hum Mutat 16: 177-178, 2000 Hishikawa N, Hashizume Y, Hirayama M, Imamura K, Washimi Y, Koike Y, Mabuchi C, Yoshida M, Sobue G: Brainstem-type Lewy body disease presenting with progressive autonomic failure and lethargy. Clin Auton Res 10: 139-143, 2000 Misu K, Hattori N, Ando Y, Ikeda S, Sobue G: Anticipation in early-but not late onset familial amyloid polyneurolathy (TTR Met 30) in Japan. Neurology 55: 451-452, 2000 Terao S, Miura N, Osano Y, Takatsu S, Mitsuma T, Sobue G: Cerebellar infarction in the territory of the medial branch of the posterior inferior cerebellar artery. J Aichi Med Univ Assoc 28: 83-88, 2000 Terao S, Miura N, Takeda A, Takahashi A, Miura T, Sobue G: Course and distribution of facial corticobulbar tract fibres in the lower brain stem. J Neurol Neurosurg Psychiat 69: 262-265, 2000 Inukai A, Doyu M, Kato T, Liang Y, Kuru S, Yamamoto M, Kobayashi Y, Sobue G: Reduced expression of DMAHP/SIX5 gene in myotonic dystrophy msucle. Muscle Nerve 23: 1421-1426, 2000 Terao S, Osano Y, Fukuona T, Miura N, Mitsuma T, Sobue G: Coexisting vertical and horizontal one and a half syndrome. J Neurol Neurosurg Psychiat 69: 401-402, 2000 Koike H, Misu K, Yasui K, Kameyama T, Ando T, Yanagi T, Sobue G: Differential response to corticosteroid therapy of MRI findings and clinical manifestations in spinal cord sarcoidosis. J Neurol 247: 544-549, 2000 Kato T, Tanaka F, Yoshida E, Indo T, Watanabe H, Yoshihara T, Doyu M, Sobue G: Sisters homozygous for the spinocerebellar associated with different clinical phenotype. Clin Genet 58: 69-73, 2000 Liang Y, Inukai A, Kuru S, Kato T, Doyu M, Sobue G: The role of lymphotoxin in pathogenesis of polymyositis. Acta Neuropathol 100: 521-527, 2000 Sawada K, Doyu M, Tanaka F, Sobue G, Kato K: Detection of triplet repeat expansion in the human genome by use of hybridization signal intensity. Anal Biochem 286: 59-66, 2000 Ito Y, Yamamoto M, Li M, Mitsuma N, Tanaka F, Doyu M., Suzumura A, Mitsuma T, Sobue G: Temporal expression of mRNAs for neuropoietic cytokine, interleukin-11 (IL-11), oncostatin M (OSM), cardiotrophin-1 (CT-1) and their receptors (IL-11Rα and OSMRb) in peripheral nerve injury. Neurochem Res 25: 1113-1118, 2000 Watanabe H, Tanaka F, Doyu M, Riku S, Yoshida M, Hashizume Y, Sobue G: Differential somatic CAG repeat instability in variable brain cell lineage in dentatorubral pallidoluysian atrophy (DRPLA): a laser-captured microdissection (LCM)-based analysis. Hum Genet 107: 452-457, 2000 Ishigaki S, Niwa J, Yoshihara T, Mitsuma N, Doyu M, Sobue G: Two novel genes, human neugrin and mouse m-neugrin, are upregulated with neuronal differentiation in neuroblastoma cells. Biochem Biophys Res Commun 279: 526-533, 2000 McCampbell A, Taylor JP, Taye AA, Robitschek J, Li M, Walcott J, Merry D, Chai Y, Paulson H, Sobue G, Fischbeck KH: CREB-binding protein sequestration by expanded polyglutamine. Hum Mol Genet 9: 2197-2202, 2000 Misu K, Yoshihara T, Shikama Y, Awaki E, Yamamoto M, Hattori N, Hirayama M, Takegami T, Nakashima K, Sobue G: An axonal form of Charcot-Marie-Tooth disease showing distinctive features in association with mutations in the periheral myelin protein zero gene (Thr124Met or Asp75Val). J Neurol Neurosurg Psychiat 69: 806-811, 2000 Ito Y, Yamamoto M, Mitsuma N, Li M, Hattori N, Sobue G: Expression of mRNAs for ciliary neurotrophic factor (CNTF), leukemia inhibitory factor (LIF), interleukin-6 (IL-6), and their receptors (CNTFRα, LIFRβ, IL-6Rα, and gp130) in human peripheral neuropathies. Neurochem Res 26: 51-58, 2001 Takeda A, Wakai M, Niwa H, Dei R, Yamamoto M, Li M, Goto Y, Yasuda T, Nakagomi Y, Watanabe M, Inagaki T, Yasuda Y, Miyata T, Sobue G: Neuronal and glial advanced glycation end product ｢Nε-(carboxymethl)lysine｣n Alzheimer’s disease brains. Acta Neuropathol 101: 27-35, 2001 Doyu M, Sawada K, Mitsuma N, Niwa J, Yoshimoto M, Fujii Y, Sobue G, Kato K: Gene expression profile in Alzheimer’s brain screened by molecular indexing. Mol Brain Res 87: 1-11, 2001 Qiao S, Iwashita T, Furukawa T, Yamamoto M, Sobue G, Takahashi M: Differential effects of LAR on biochemical and biological activities of RET-MEN2A and RET-MEN2B mutant protein. J Biol Chem 276: 9460-9467, 2001 Hattori N, Misu K, Koike H, Ichimura M, Nagamatsu M, Hirayama M, Sobue G: Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci 184: 57-63, 2001 Niwa J, Ishigaki S, Doyu M, Suzuki T, Tanaka K, Sobue G: A novel centrosomal RING-finger protein, Dorfin, mediates ubiquitin ligase activity. Biochem Biophys Res Commun 281: 706-713, 2001 Yoshihara T, Kanda F, Yamamoto M, Ishihara H, Misu K, Hattori N, Chihara K, Sobue G: A novel missense mutation in the early growth response 2 gene associated with late-onset Charcot-Marie-Tooth disease type 1. J Neurol Sci 184: 149-153, 2001 Ochi K, Nozaki H, Tanaka F, Kato S, Fukuzawa R, Sobue G, Fukuchi Y, Toyama Y, Hata J, Umezawa A: Specific bisulfite modification of CTG triplet repeats of the androgen receptor gene, a gene associated with the triplet repeat disease. X-linked spinal and bulbar muscular atrophy (Kennedy disease). Neurosci Res Commun 28: 1-10, 2001 Adachi H, Kume A, Li M, Nakagami Y, Niwa H, Do J, Sang C, Kobayashi Y, Doyu M, Sobue G: Transgenic mice with an expande CAG repeat controlled by the human AR promoter show polyglutamine nuclear inclusions and neuronal dysfunction without neuronal cell death. Hum Mol Genet 10: 1039-1048, 2001 Watanabe H, Ieda T, Katayama T, Takeda A, Aiba I, Doyu M, Hirayama M, Sobue G: Cardiac I-meta-iodobenzylguanidine (MIBG) uptaken in dementia with Lewy bodies: comparison with Alzheimer’s disease. J Neurol Neurosurg Psychiat 70: 781-783, 2001 Yamamoto M, Ito Y, Mitsuma N, Li M, Hattori N, Sobue G: Pathology-related differential expression regulation of NGF, GDNF, CNTF, and IL-6 mRNAs in human vascultic neuropathy. Muscle Nerve 24: 830-833, 2001 Koike H, Mori K, Misu K, Hattori N, Ito H, Hirayama M, Sobue G: Painful alcoholic polyneuropathy with predominant small-fiber loss and normal thiamine status. Neurology 56: 1727-1732, 2001 Koike H, Misu K, Hattori N, Ito S, Ichimura M, Ito H, Hirayama M, Nagamatsu M, Sasaki I, Sobue G: Postgastrectomy polyneuropathy with thiamine deficiency. J Neurol Neurosurg Psychiat 71: 357-362, 2001 Yamamoto M, Li M, Mitsuma N, Ito S, Kato M, Takahashi M, Sobue G: Preserved phosphorylation of RET receptor protein in spinal motor neurons of patients with amyotrophic lateral sclerosis: an immunohistochemical study by a phosphorylation-specific antibody at tyrosine 1062. Brain Res 912: 89-94, 2001 Mori K, Koike H, Misu K, Hattori N, Ichimura M, Sobue G: Spinal cord magnetic resonance imaging demonstrates sensory neuronal involvement and clinical severity in neuronopathy associated with Sjögren’s syndrome. J Neurol Neurosurg Psychiat 71: 488-492, 2001 Watanabe H, Misu K, Hirayama M, Hattori N, Yoshihara T, Doyu M, Sobue G: Low cardiac 123I-MIBG uptake in lateonset familial amyloid polyneuropathy type I (TTR Met 30). J Neurol 248: 627-629, 2001 Hashimoto Y, Niikura T, Tajima H, Yasukawa T, Sudo H, Ito Y, Kita Y, Kawasumi M, Kouyama K, Doyu M, Sobue G, Koide T, Tsuji S, Lang J, Kurokawa K, Nishimoto I: A rescue factor abolishing neuronal cell death by a wide spectrum of familial Alzheimer’s disease genes and Aβ. Proc Natl Acad Sci USA 98: 6336-6341, 2001 Hishikawa N, Hashizume Y, Yoshida M, Sobue G: Widespread occurrence of argyrophilic glial inclusions in Parkinson’s disease. Neuropathol Appl Neurobiol 27: 362-337, 2001 Yoshihara T, Ishigaki S, Yamamoto M, Liang Y, Niwa J, Takeuchi H, Doyu M, Sobue G: Differential expression of inflammation-and apoptosis-related genes in spinal cords of a mutant SOD1 trangenic mouse model of familial amyotrophic lateral sclerosis. J Neurochem 80: 158-167, 2002 Yamamoto M, Kobayashi Y, Li M, Niwa H, Mitsuma N, Ito Y, Mutamatsu T, Sobue G: In Vivo gene electroporation of glial cell line-derived neurotrophic factor (GDNF) into skeletal muscle of SOD1 mutant mice. Neurochem Res 26: 1201-1207, 2002 Watanabe H, Misu K, Kobayashi T, Hattori N, Doyu M, Yokoyama I, Ando Y, Nakao A, Sobue G: ABO-incompatible auxiliary partial orthotopic liver transplant for late-onset familial amyloid polyneuropathy. J Neurol Sci 195: 63-66, 2002 Katsuno M, Ando T, Hakusui S, Yanagi T, Sobue G: Motor conduction studies in miller fisher syndrome with severe tetraparesis. Muscle Nerve 25: 378-382, 2002 Yamamoto M, Ito Y, Mitsuma N, Li M, Hattori N, Sobue G: Parallel expression of neurotrophic factors and their receptors in chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 25: 601-604, 2002 Mori K, Hattori N, Sygiura M, Koike H, Misu K, Ichimura M, Hirayama M, Sobue G: Chronic inflammatory demyelinating polyneuropathy presenting with features of GBS. Neurology 58: 979-982, 2002 Ikeda S, Nakazato M, Ando Y, Sobue G: Familial transthyretin-type amyloid polyneuropathy in Japan. Neurology 58: 1001-1007, 2002 Watanabe H, Saito Y, Terao S, Ando T, Kachi T, Mukai E, Aiba K, Abe Y, Tamakoshi A, Doyu M, Hirayama M, Sobue G: Progression and prognosis in multiple system atrophy. An analysis of 230 Japanese patients. Brain 125: 1070-1083, 2002 Kato M, Takeda K, Kawamoto Y, Iwashita Akhand AA, Senga T, Yamamoto M, Sobue G, Hamaguchi M, Takahashi M, Nakashima I: Repair by src kinase of function-impaired RET with multiple endocrine neoplasia type 2A mutation with substitutions of tyrosines in the COOH-terminal kinase domain for phenylalanine. Cancer Res 62: 2414-2422, 2002 Iwai K, Yamamoto M, Yoshihara T, Sobue G: Anticipation in familial amyotrophic lateral sclerosis with SOD1-G93S mutation. J Neurol Neurosurg Psychiat 72: 819-820, 2002 Dei R, Takeda A, Niwa H, Li M, Nakagomi Y, Watanabe M, Inagaki T, Washimi Y, Yasuda Y, Horie K, Toshio M, Sobue G: Lipid peroxidation and advanced glycation and products in the brain in normal aging and in Alzheimer’s disease. Acta Neuropathol 104: 113-122, 2002 Hattori N, Mori K, Misu K, Koike H, Ichimura M, Sobue G: Mortality and morbidity in peripheral neuropathy associated Churg-Strauss syndrome and microscopic polyangiitis. J Rheumatol 29: 1408-1414, 2002 Ishigaki S, Liang Y, Yamamoto M, Niwa J., Ando Y, Yoshihara T, Takeuchi H., Doyu M, Sobue G: X-linked inhibitor of apoptosis protein is involved in mutant SOD-1 mediated neuronal degeneration. J Neurochem 82: 576-584, 2002 Katsuno M, Adachi H, Kume A, Li M, Nakagomi Y, Niwa H, Sang C, Kobayashi Y, Doyu M, Sobue G: Teststerone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Neuron 35: 843-854, 2002 Niwa H, Hayakawa K, Yamamoto M, Itoh T, Mitsuma T, Sobue G: Differential age-dependent trophic responses of nodose, sensory, and sympathetic neurons to neurotrophins and GDNF: potencies for neurite extension in explant culture. Neurochem Res 27: 485-496, 2002 Kato T, Indo T, Yoshida E, Iwasaki Y, Sone M, Sobue G: Contrast-enhanced 2D cine phase MR angiography for measurement of basilar artery blood flow in posterior circulation ischemia. Am J Neuroradiol 23: 1346-1351, 2002 Niwa J, Ishigaki S, Hishikawa N, Yamamoto M, Doyu M, Murata S, Tanaka K, Taniguchi N, Sobue G: Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity. J Biol Chem 277: 36793-36798, 2002 Takeuchi H, Kobayashi Y, Yoshihara T, Niwa J, Doyu M, Ohtsuka K, Sobue G: Hsp70 and Hsp40 improve neurite outgrowth and suppress intracytoplasmic aggregate formation in cultured neuronal cells expressing mutant SOD1. Brain Res 949: 11-22, 2002 Takeuchi H, Kobayashi Y, Ishigaki S, Doyu M, Sobue G: Mitochondrial localization of mutant superoxide dismutase 1 triggers caspase-depenent cell death in a cellular model of familial amyotrophic lateral sclerosis. J Biol Chem 277: 50966-50972, 2002 Sang C, Kobayashi Y, Du J, Katsuno M, Adachi H, Doyu M, Sobue G: c-Jun N-terminal kinase pathway mediates lactacustin-induced cell death in a neuronal differentiated neuro2a cell line. Mol Brain Res 108: 7-17, 2002 Ishigaki S, Niwa J, Ando Y, Yoshihara T, Sawada K, Doyu M, Yamamoto M, Kato K, Yotsumoto Y, Sobue G: Differentially expressed genes in sporadic amyotrophic lateral sclerosis spinal cords -screening by molecular indexing and subsequent cDNA microarray analysis. FEBS Lett 531: 354-358, 2002 Koike H, Misu K, Ikeda S, Ando Y, Nakazato M, Ando E, Yamamoto M, Hattori N, Sobue G: Type I (transthyretin Met30) familial amyloid polyneuropathy in Japan: early- vs late-onset form. Arch Neurol 59: 1771-1776, 2002 Ito Y, Kato T, Suzuki T, Yokokawa Y, Aiba I, Arahata Y, Ito E, Ito K, Yasuda T, Sobue G: Neuroradiologic and clinical abnormalities in dementia of diffuse neurofibrillary tangles with calcification (Kosaka-Shibayama disease). J Neurol Sci 209: 105-109, 2002 Kuru S, Inukai A, Kato T, Liang Y, Kimura S, Sobue G: Expression of tumor factor-α in regenerating muscle fibers in inflammatory and non-inflammatory myopathies. Acta Neuropathol 105: 217-224, 2003 Kobayashi R, Koike Y, Hirayama M, Ito H, Sobue G: Skin sympathetic nerve function sleep-a study with effector responses. Anton Neurosci 103: 121-126, 2003 Hattori N, Yamamoto M, Yoshihara T, Koike H, Nakagawa N, Yoshikawa H, Ohnishi A, Hayasaka K, Onodera O, Baba M, Yasuda H, Saito T, Nakashima K, Kira J, Kaji R, Oka N, Sobue G, the Study Group for Hereditary Neuropathy in Japan: Demyelinating and axonal features of Charcot-Marie-Tooth disease with mutations of myelin-related proteins (PMP22, MPZ and Cx32): a clinicopathological study of 205 Japanese patients. Brain 126: 134-151, 2003 Hishikawa N, Hashizume Y, Yoshida M, Sobue G: Clinical and neuropathological correlates of Lewy body disease.  Acta Neuropathol 105: 341-350, 2003 Yamada T, Nakamura A, Horibe K, Washimi Y, Bundo M, Kato T, Ito K, Kachi T, Sobue G: Asymmetrical enhancement of middle-latency auditory evoked fields with aging. Neurosci Lett 337: 21-24, 2003 Hamada K, Hirayama M, Watanabe H, Kobayashi R, Ito H, Ieda T, Koike Y, Sobue G: Onset age and severity impairment are associated with reduction of myocardial 123I-MIBG uptake in Parkinson’s disease. J Neurol Neurosurg Psychiat 74: 423-426, 2003 Adachi H, Katsuno M, Minamiyama M, Sang C, Pagoulatous G, Angelidis C, Kusakabe M, Yoshiki A, Kobayashi Y, Doyu M, Sobue G: Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein. J Neurosci 23: 2203-2211, 2003 Hirayama M, Fukatsu H, Watanabe H, Koike Y, Noda A, Ito H, Kobayashi R, Sobue G: Sequential constriction of upper airway and vocal cords in sleep apnoea of multiple system atrophy: low field magnetic resonance fluoroscopic study. J Neurol Neurosurg Psychiat 74: 642-645, 2003 Abe Y, Kachi T, Arahata Y, Yamada T, Washimi Y, Iwai K, Ito K, Yanagisawa N, Sobue G: Occipital hypoperfusion in Parkinson’s disease without dementia: correlation to impaired cortical visual processing. J Neurol Neurosurg Psychiat 74: 419-422, 2003 Koike H, Iijima M, Sugiura M, Mori K, Hattori N, Ito H, Hirayama M, Sobue G: Alcoholic neuropathy is clinicopathologically distinct from thiamine-deficiency neuropathy. Ann Neurol 54: 19-29, 2003 Hishikawa N, Hashizume Y, Yoshida M, Sobue G: Reply to K. Jellinger. Acta Neuropathol 106: 109, 2003 Katsuno M, Adachi H, Doyu M, Minamiyama M, Sang C, Kobayashi Y, Inukai A, Sobue G: Leuprorelin rescue polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy. Nat Med 9: 768-773, 2003 Hishikawa N, Niwa J, Doyu M, Ito T, Ishigaki S, Hashizume Y, Sobue G: Dorfin localizes to the ubiquitilated inclusions in Parkinson’s disease, dementia with Lewy bodies, multiple system atrophy, and amyotrophic lateral sclerosis. Am J Pathol 163: 609-619, 2003 Hishikawa N, Hashizume Y, Ujihara N, Okada Y, Yoshida M, Sobue G: α-Synuclein-positive structures in associations with diffuse neurofibrillary tangles with calcification. Neuropathol Appl Neurobiol 29: 280-287, 2003 Ishihara K, Yamagishi N, Saito Y, Adachi H, Kobayashi Y, Sobue G, Ohtsuka K, Hatayama T: Hsp 105α suppresses the aggregation of truncated androgen receptor with expanded CAG repeats and cell toxicity. J Biol Chem 278: 25143-25150, 2003 Ito T, Niwa J, Hishikawa N, Ishigaki S, Doyu M, Sobue G: Dorfin localizes to Lewy bodies and ubiquitylates synphilin-1. J Biol Chem 278: 29106-29114, 2003 Mori K, Iijima M, Sugiura M, Koike H, Hattori N, Ito H, Hirayama M, Sobue G: Sjögren’s syndrome associated painful sensory neuropathy without sensory ataxia. J Neurol Neurosurg Psychiat 74: 1320-1322, 2003 Ando Y, Liang Y, Ishigaki S, Niwa J, Jiang Y, Kobayashi Y, Yamamoto M, Doyu M, Sobue G: Caspase-1 and -3 are differentially upregulated in motor neurons and glial cells in mutant SOD1 transgenic mouse spinal cord: a study usign laser microdissection and real time RT-PCR. Neurochem Res 28: 839-846, 2003 Katsuno M, Adachi H, Inukai A, Sobue G: Transgenic mouse models of spinal and bulbar muscular atrophy (SBMA). Cytogenet Genome Res 100: 243-251, 2003 Sobue G, Koike H, Misu K, Hattori N, Yamamoto M, Ikeda S, Ando Y, Nakazato M, Inukai A: Clinicopathologic and genetic features of early- and late-onset FAP type I (FAP ATTR Val30Met) in Japan. Amyloid 1: 32-38, 2003 Nodera H, Bostock H, Kuwabara S, Sakamoto T, Asanuma K, Jia-Ying S, Ogawara K, Hattori N, Hirayama M, Sobue G, Kaji R: Nerve excitability properties in Charcot-Marie-Tooth disease type 1A. Brain 127: 203-211, 2003 Wada M, Kimura M, Daimon M, Kurita K, Kato T, Johmura Y, Johkura K, Kuroiwa Y, Sobue G: An unusual phenotype of Mcleod syndrome with late onset axonal neuropathy. J Neurol Neurosurg Psychiat 74: 1697-1699, 2003 Watanabe H, Fukatsu H, Katsuno M, Sugiura M, Hamada K, Okada Y, Hirayama M, Ishigkai T, Sobue G: Multiple regional 1H-MR spectroscopy in multiple system atrophy: NAA/Cr reduction in pontine base as a valuable diagnostic marker. J Neurol Neurosurg Psychiat 75: 103-109, 2004 Watanabe H, Fukatsu H, Hisikawa N, Hashizume Y, Sobue G: Field strengths and sequences influence putaminal MRI findings in multiple system atrophy. Neurology 62: 671, 2004 Yamamoto M, Yoshihara T, Hattori N, Sobue G: Glu528del in NEFL is a polymorphic variant rather than a disease-causing mutant for Charcot-Marie-Tooth disease in Japan. Neurogenetics 5: 75-77, 2004 Konagaya M, Matsumoto A, Takase S, Mizutani T, Sobue G, Konishi T, Hayabara T, Iwashita H, Ujihira T, Miyata T, Matsuoka Y: Clinical analysis of longstanding substance myelo-optico-neuropathy: sequelae of clioquinol at 32 years after its ban. J Neurol Sci 218: 85-90, 2004 Takeuchi H, Niwa J, Hishikawa N, Ishigaki S, Tanaka F, Doyu M, Sobue G: Dorfin prevents cell death by reducing mitochondrial localiaing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis. J Neurochem 89: 64-72, 2004 Yamada S, Matsuo K, Hirayama M, Sobue G: The effects of levodopa on apraxia of lid opening: A case report. Neurology 62: 830-831, 2004 Terao S, Miura N, Osano Y, Noda A, Sobue G: Rapidly progressive fatal respiratory failure (Ondine’s curse) in the lateral medullary sundrome. J Stroke Cerebrovasc Dis 13: 41-44, 2004 Minamiyama M, Katsuno M, Adachi H, Waza M, Sang C, Kobayashi Y, Tanaka F, Doyu M, Inukai A, Sobue G: Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Hum Mol Genet 13: 1183-1192, 2004 Ohmori H, Ando Y, Makita Y, Onouchi Y, Nakajima T, Saraiva MJM, Terazaki H, Suhr O, Sobue G, Nakamura M, Yamaizumi M, Munar-Ques M, Inoue I, Uchino M, Hata A: Common origin of the Val30Met mutatioin responsible for the amyloidogenic transthyretin type of familial amyloidotic polyneuropathy. J Med Genet 41: 1-5, 2004 Koike H, Misu K, Sugiura M, Iijima M, Mori K, Yamamoto M, Hattori N, Mukai E, Ando Y, Ikeda S, Sobue G: Pathology of early- vs late-onset TTR Met30 familial amyloid polyneuropathy. Neurology 63: 129-138, 2004 Kurihara S, Adachi Y, Imai C, Araki H, Hattori N, Numakura C, Lin Y, Hayasaka K, Sobue G, Nakashima K: Charcot-Marie-Tooth families in Japan with MPZ Thr124Met mutation. J Neurol Neurosurg Psychiat 75: 1492-1494, 2004 Mitsuma N, Yamamoto M, Iijima M, Hattori N, Ito Y, Tanaka F, Sobue G: Wide range of lineages of cells expressing nerve growth factor mRNA in the nerve lesions of patients with vasculitic neuropathy: An implication of endoneurial macrophage for nerve regeneration. Neuroscience 129: 109-117, 2004 Mabuchi N, Watanabe H, Atsuta N, Hirayama M, Ito H, Fukatsu H, Kato T, Ito K, Sobue G: Primary lateral sclerosis presenting parkinsonian symptoms without nigrostriatal involvement. J Neurol Neurosurg Psychiat 75: 1768-1771, 2004 Ishigaki S, Hishikawa N, Niwa J, Iemura S, Natsume T, Hori S, Kakizuka A, Tanaka K, Sobue G: Physical and functional interaction between Dorfin and Valosin-containing protein that are colocalized in ubiquitylated inclusions in neurodegenerative disorders. J Biol Chem 279: 51376-51385, 2004 Koike H, Iijima M, Mori K, Hattori N, Ito H, Hirayama M, Sobue G: Postgastrectomy polyneuropathy with thiamine deficiency is identical to beriberi neuropathy. Nutrition 20: 961-966, 2004 Okada Y, Shimazaki T, Sobue G, Okano H: Retinoic-acid-concentration-dependent acquisition of neural cell identity during in vitro differentiation of mouse embryonic stem cells. Dev Biol 275: 124-142, 2004 Jiang YM, Yamamoto M, Kobayashi Y, Yoshihara T, Liang Y, Terao S, Takeuchi H, Ishigaki S, Katsuno M, Adachi H, Niwa J, Tanaka F, Doyu M, Yoshida M, Hashizume Y, Sobue G: Gene expression profile of motor neurons in sporadic amyotrophic lateral sclerosis. Ann Neurol 57: 236-251, 2005 Adachi H, Katsuno M, Minamiyama M, Waza M, Sang C, Nakagomi Y, Kobayashi Y, Tanaka F, Doyu M, Inukai A, Yoshida M, Hashizume Y, Sobue G: Widespread nuclear and cytoplasmic mutant androgen receptor accumulation in spinal and bulbar muscular atrophy. Brain 128: 659-670, 2005 Iijima M, Yamamoto M, Hirayama M, Tanaka F, Katsuno M, Mori K, Koike H, Hattori N, Arimura K, Nakagawa M, Yoshikawa H, Hayasaka K, Onodera O, Baba M, Yasuda H, Saito T, Nakazato M, Nakashima K, Kira J, Kaji R, Oka N, Sobue G: Clinical and electrophysiologic correlates of IVIg responsiveness in CIDP. Neurology 64: 1471-1475, 2005 水野　美邦　(順天堂大学・医学部・脳神経内科) Lu CS, Wu JC, Tsai CH, Chen RS, Chou WYH, Hattori N, Yoshino H, Mizuno, Y: Clinical and genetic studies on familial parkinsonism: The first report on a parkin gene mutation in a Taiwanese family. Mov Disord 16: 164-166, 2001 Imai Y, Soda M, Inoue H, Hattori N, Mizuno Y, Takahashi R: An unfolded putative transmembrane polypeptide, which can lead to endoplasmic reticulum stress, is a substrate of parkin. Cell 105: 891-902, 2001 Jeon BS, Kim JM, Lee DS, Hattori N, Mizuno Y: An apparently sporadic case with parkin gene mutation in a Korean woman. Arch Neurol 58: 988-989, 2001 Nisipeanu P, Inzelberg R, Abo Mouch S, Carasso RL, Blumen SC, Zhang J, Matsumine H, Hattori N, Mizuno Y: Parkin gene causing benign autosomal recessive juvenile parkinsonism. Neurology 56: 1573-1577, 2001 Mellick GD, Buchanan DD, Hattori N, Brookes AJ, Mizuno Y, Le Couteur DG, Silburn PA: The parkin gene S/N167 polymorphism in Australian Parkinson's disease patients and controls. Parkinsonism Relat Disord 7: 89-91, 2001 Mogi M, Togari A, Kondo T, Mizuno Y, Kogure O, Kuno S, Ichinose H, Nagatsu T: Glial cell line-derived neurotrophic factor in the substantia nigra from control and parkinsonian brains. Neurosci Lett 300: 179-181, 2001 Kubo S, Kitami T, Noda S, Shimura H, Uchiyama Y, Asakawa S, Minoshima S, Shimizu N, Mizuno Y, Hattori N: Parkin is associated with cellular vesicles. J Neurochem 78: 42-54, 2001 Miwa H, Kagohashi M, Noda K, Miyashita N, Tanaka S, Mizuno Y: Eyelid motor extinction. J Neurol 248: 343-344, 2001 Miwa H, Fuwa T, Nishi K, Kondo T: Subthalamo-pallido-striatal axis: a feedback system in the basal ganglia. NeuroReport 12: 3795-3798, 2001 Miwa H, Mizuno Y: Capgras syndrome in Parkinson's disease. J Neurol 248: 804-805, 2001 Miwa H, Furuya T, Tanaka S, Mizuno Y: Neurosarcoidosis associated with interferon therapy. Eur Neurol 45: 288-289, 2001 Miwa H, Miwa T, Imai H, Mizuno Y: Obsessive compulsive disorder-like behavioral changes in pure akinesia. Parkinsonism Relat Disord 7: 315-317, 2001 Miwa H, Iijima M, Tanaka S, Mizuno Y: Generalized convulsions after consuming a large amount of Gingko nuts. Epilepsia 42: 280-281, 2001 Noda K, Okuma Y, Fukae J, Fujishima K, Sadamasa H, Yoshiike T, Mizuno Y: Sweet’s syndrome associated with encephalitis. J Neurol Sci 188: 95-97, 2001 Noda K, Miwa H, Miyashita N, Tanaka S, Mizuno Y: Monoataxia of upper extremity in motor cortical infarction. Neurology 56: 1418-1419, 2001 Mochizuki H, Hayakawa H, Migita M, Shibata M, Tanaka R, Suzuki A, Shimo-Nakanishi Y, Urabe T, Yamada M, Tamayose K, Shimada T, Miura M, Mizuno Y: An AAV-derived apaf-1 dominant negative inhibitor prevents MPTP toxicity as antiapoptotic gene therapy for Parkinson’s disease. Proc Natl Acad Sci USA 98: 10918-10923, 2001 Mori H, Motoi Y, Kobayashi T, Hasegawa M, Yamamura A, Iwatsubo T, Mizuno Y: Tau accumulation in a patient with pallidonigroluysian atrophy. Neurosci Lett 309: 89-92, 2001 Nohara C, Akiba H, Nakajima A, Inoue A, Koh CS, Ohshima H, Yagita H, Mizuno Y, Okumura Y: Amelioration of experimental autoimmune encephalomyelitis with anti-OX40 ligand monoclonal antibody: A critical role for OX40 ligand in migration, but not development, of pathogenic T cells. J Immunol 166: 2108-2115, 2001 Okuma Y, Tanaka R, Fujishima K, Kobayashi T, Mizuno Y: Cortical reflex action myoclonus in neurosyphilis. Eur Neurol 45: 193-194, 2001 Okuma Y, Mizuno Y: Myoclonic tremor in patients with parkinsonian-type multiple system atrophy. Mov Disord 16: 378-379, 2001 Okuma Y, Fujishima K, Machida Y, Inagaki N, Sugita Y, Mizuno Y: Propriospinal negative myoclonus. Eur Neurol 46: 99-101, 2001 Shimo-Nakanishi Y, Urabe T, Hattori N, Watanabe Y, Nagao T, Yokochi M, Hamamoto M, Mizuno Y: Polymorphism of the lipoprotein lipase gene and risk of atherothrombotic cerebral infarction in the Japanese. Stroke 32: 1481-1486, 2001 Shimura H, Schlossmacher MG, Hattori N, Frosch MP, Trockenbacher A, Schneider R, Mizuno Y, Kosik KS, Selkoe DJ: Ubiquitination of a new form of α-synuclein by parkin from human brain: Implications for Parkinson's disease. Science 293: 263-269, 2001 Takanashi M, Mochizuki H, Yokomizo K, Hattori N, Mori H, Yamamura Y, Mizuno Y: Iron accumulation in the substantia nigra of autosomal recessive juvenile parkinsonism (ARJP). Parkinsonism Relat Disord 7: 311-314, 2001 Tanaka K, Suzuki T, Chiba T, Shimura H, Hattori N, Mizuno Y: Parkin is linked to the ubiquitin pathway. J Mol Med 79: 482-494, 2001 Wang M, Suzuki T, Kitada T, Asakawa S, Minoshima S, Shimizu N, Tanaka K, Mizuno Y, Hattori N: Developmental changes in the expression of parkin and UbcR7, a parkin-interacting and ubiquitin-conjugating enzyme, in rat brain. J Neurochem 77: 1561-1568, 2001 Hyun DH, Lee M, Hattori N, Kubo S, Mizuno Y, Halliwell B, Jenner P: Effect of wild-type or mutant Parkin on oxidative damage, nitric oxide, antioxidant defenses, and the proteasome. J Biol Chem 277: 28572-28577, 2002 Kobayashi T, Mori H, Okuma Y, Dickson DW, Cookson N, Tsuboi Y, Motoi Y, Tanaka R, Miyashita N, Anno M, Narabayashi H, Mizuno Y: Constrasting genotypes of the tau gene in two phenotypically distinct patients with P301L mutation of frontotemporal dementia and parkinsonism linked to chromosome 17. J Neurol 249: 669-675, 2002 Miwa H, Kajimoto M, Kondo T: Chorea in motor neuron disease. Move Disord (letter) 17: 1397, 2002 Miwa H, Kondo T, Mizuno Y: Bell's palsy-induced blepharospasm. J Neurol 249: 452-454, 2002 Miwa H, Mizuno Y: Enlargements of somatosensory-evoked potentials in progressive supranuclear palssy. Acta Neurol Scand 106: 209-212, 2002 Miwa H, Mizuno Y, Kondo T: Familial hemifacial spasm: report of cases and review of literature. J Neurol Sci 193: 97-102, 2002 Mori H, Oda M, Komori T, Arai N, Takanashi M, Mizutani T, Hirai S, Mizuno Y: Lewy bodies in progressive supranuclear palsy. Acta Neuropathol 104: 273-278, 2002 Ohizumi H, Okuma Y, Fukae J, Fujishima K, Goto K, Mizuno Y: Head tremor in dentatorubral-pallidoluysian atrophy. Acta Neurol Scand 106: 319-321, 2002 Okuma Y, Mizuno Y, Lee RG: Reciprocal Ia inhibition in patients with asymmetric spinal spasticity. Clin Neurophysiol 113: 292-297, 2002 Schossmacher MG, Frosch MP, Ping-Gai W, Medina M, Sharma N, Forno L, Ochiishi T, Shimura H, Sharon R, Hattori N, Langston JW, Mizuno Y, Hyman BT, Selkoe DJ, Kosik K: Parkin localizes to the Lewy bodies of Parkinson disease and dementia with Lewy bodies. Am J Pathol 160: 1655-1667, 2002 Suzuki A, Obi K, Urabe T, Hayakawa H, Yamada M, Kaneko S, Onodera M, Mizuno Y, Mochizuki H: Feasibility of ex vivo gene therapy for neurological disorders using the new retroviral vector GCDNsap packaged in the vesicular stomatitis virus G protein. J Neurochem 82: 953-960, 2002 Takanashi M, Mori H, Arima K, Mizuno Y, Hattori N: Expression pattern of tau mRNA isoforms correlates with susceptible lesions in progressive supuranuclear palsy and corticobasal degeneration. Mol Brain Res 104: 210-219, 2002 Takanashi M, Ohta S, Matsuoka S, Mori H, Mizuno Y: Mixed multiple system atrophy and progressive supranuclear palsy: a clinical and pathological report of one case. Acta Neuropathol 103: 82-87, 2002 Tanaka M, Fuku N, Takeyasu T, Guo L-J, Hirose R, Kurata M, Borgeld HJ, Yamada Y, Maruyama W, Arai Y, Hirose N, Oshida Y, Sato Y, Hattori N, Mizuno Y, Iwata S, Yagi K: Golden mean to longevity: Rareness of mitochondrial cytochrome b variants in centenarians but not in patients with Parkinson’s disease. J Neurosci Res 70: 347-355, 2002 Tanaka R, Mochizuki H, Suzuki A, Ishitani R, Katsube N, Mizuno Y, Urabe T: Induction of glyceraldehyde-3-phosphate dehydrogenase (GAPDH) expression in rat brain after focal ischemia/reperfusion. J Cereb Blood Flow Metab 22: 280-288, 2002 Tsuboi Y, Baker M, Hutton ML, Uitti RJ, Rascol O, Delisle MB, Soulages X, Murrell JR, Ghetti B, Yasuda M., Komure O. Kuno S, Arima K, Sunohara N, Kobayashi T, Mizuno Y, Wszolek ZK: Clinical and genetic studies of families with the tau N279K mutation (FTDP-17). Neurology 59: 1791−1793, 2002 Urabe T, Tanaka R, Noda K, Mizuno Y: Anticoagulant therapy with a selective thrombin inhibitor for acute cerebral infarction: usefulness of coagulation markers for evaluation of efficacy. J Thromb Thrombolysis 13: 153-158, 2002 Furuya T, Tanaka R, Urabe T, Hayakawa J, Yamada M, MigitaM, Shimada T, MizunoY, Mochizuki H: Establishment of modified chimeric mice using GFP bone marrow as a model for neurological disorders NeuroReport 14: 629-631, 2003 Gouider-Khouja Ｎ, Larnaout A, Amouri R, Sfar S, Belal S, Hamida CB, Hamida MB, Hattori N, Mizuno Y, Hentati F: Autosomal recessive parkinsonism linked to parkin gene in Tunisian family. Clinical, genetic and pathological study. Parkinsonism Relat Disord 9: 247-251, 2003 Hatano T, Okuna Y, Iijima M, Fujishima K, Goto K, Mizuno Y: Cervical dystonia in dentatorubral-pallidoluysian atrophy. Acta Neurol Scand 108: 287-289, 2003 Inzelberg R, Hattori N, Nisipeanu P, Abo Mouch S, Blumen SC, Carasso RL, Mizuno Y: Camptocormia, axial dystonia, and parkinsonism: Phenotypic heterogeneity of a parkin mutation. Neurology 60: 1393-1394, 2003 Kim SJ, Sung JY, Um JW, Hattori N, Mizuno Y, Tanaka K, Paik SR, Kim J, Chung KC: Parkin cleaves intracellular alpha-synuclein inclusions via the activation of calpain. J Biol Chem 278: 41890-41899, 2003 Kobayashi H, Kruger R, Markopoulou K, Wszolek Z, Chase B, Taka H, Mineki, Murayama K, Riess O, Mizuno Y, Hattori N: Haploinsufficiency at the α-synuclein gene underlies phenotypic severity in familial Parkinson's disease. Brain 126: 32-42, 2003 Kobayashi T, Matsumine H, Zhang J, Imamichi Y, Mizuno Y, Hattori N: Pseudo-autosomal dominant inheritance of PARK2: two families with parkin gene mutations. J Neurol Sci 207: 11-17, 2003 Kobayashi T, Ota S, Tanaka K, Ito Y, Hasegawa M, Umeda Y, Motoi Y, Takanashi M, Yasuhara M, Anno M, Mizuno Y, Mori H: A novel L266V mutation of the tau gene causes frontotemporal dementia with a unique tau pathology. Ann Neurol 53: 133-137, 2003 Nagano Y, Yamashita H, Takahashi T, Kishida S, Nakamura T, Iseki E, Hattori N, Mizuno Y, Kikuchi A, Matsumoto M: Siah-1 facilitates ubiquitination and degradation of synphilin-1. J Biol Chem 278: 51504-51514, 2003 Mizuno Y, Yanagisawa N, Kuno S, Yamamoto M, Hasegawa K, Origasa H, Kowa H, The Japan Pramipexole Study Group: Randomized, double-blind study of pramipexole with placebo and bromocriptine in advanced Parkinson's disease. Mov Disord 18: 1149-1156, 2003 Rawal N, Periquet M, Lohmann E, Lucking CB, Teive HA, Ambrosio G, Raskin S, Lincoln S, Hattori N, Guimaraes J, Horstink MW, Dos Santos Bele W, Brousolle E, Destee A, Mizuno Y, Farrer M, Deleuze JF, De Michele G, Agid Y, Durr A, Brice A, French Parkinson's Disease Genetics Study Group: European Consortium on Genetic Susceptibility in Parkinson's Disease. New parkin mutations and atypical phenotypes in families with autosomal recessive parkinsonism. Neurology 60: 1378-1381, 2003 Sakata E, Yamaguchi Y, Kurimoto E, Yokoyama S, Yamada S, Kawahara H, Yokosawa H, Hattori N, Mizuno Y, Tanaka K: Parkin binds the Rpn10 subunit of 26S proteasomes through its ubiquitin-like domain. EMBO Rep 4: 301-306, 2003 Shimo-Nakanishi Y, Hasebe T, Suzuki A, Mochizuki H, Nomiyama T, Tanaka Y, Nagaoka I, Mizuno Y, Urabe T: Functional effects of NAD(P)H oxidase p22(phox) C242T mutation in human leukocytes and association with thrombotic cerebral infarction. Atherosclerosis 175: 109-115, 2004 Takahashi R, Imai Y, Hattori N, Mizuno Y: Parkin and endoplasmic reticulum stress. Ann NY Acad Sci 911: 101-106, 2003 Takubo H, Harada T, Hashimoto T, Inaba Y, Kanazawa I, Kuno S, Mizuno Y, Mizuta E, Murata M, Nagatsu T, Nakamura S, Yanagisawa N, Narabayashi H: A collaborative study on the malignant syndrome in Parkinson's disease and related disorders. Parkinsonism Relat Didord 9 (Suppl 1): S31-S41, 2003 Takubo H, Shimoda SM, Mizuno Y: Serum creatine kinase is elevated in patients with Parkinson's disease: a case controlled study. Parkinsonism Relat Didord 9 (Suppl 1): S43-S46, 2003 Tanaka R, Komine KM, Mochizuki M, Yamada M, Furuya T, Migita M, Shimada T, Mizuno Y, Urabe T: Migration of enhanced green fluorescent protein expressing bone marrow-derived microglia/macrophage into the mouse brain following permanent focal ischemia. Neuroscience 117: 531-539, 2003 Tanaka R, Yamashiro K, Mochizuki H, Cho N, Onodera M, Mizuno Y, Urabe T: Neurogenesis after transient global ischemia in the adult hippocampus visualized by improved retroviral vector. Stroke 35: 1454-1459, 2004 Toda T, Momose Y, Murata M, Tamiya G, Yamamoto M, Hattori N, Inoko H: Toward identification of susceptibility genes for sporadic parkinson's disease. J Neurol 205[suppl3]: III40-III43, 2003 Arai H, Furuya T, Yasuda T, Miura M, Mizuno Y, Mochizuki H: Neurotoxic effects of lipopolysaccharide on nigral dopaminergic neurons are mediated by microglial activation, interleukin-1α, and expression of caspase-11 in mice. J Biol Chem 279: 51647-51653, 2004 Furuya T, Hayakawa H, Yamada M, Yoshimi K, Hisahara S, Miura M, Mizuno Y, Mochizuki H: Caspase-11 mediates inflammatory dopaminergic cell death in the 1-methyl-4-phenyl-1, 2, 3, 6 -tetrahydropyridine mouse model of Parkinson’s disease. J Neurosci 24: 1865-1872, 2004 Hatano Y, Li Y, Sato K, Asakwa S, Yamamura Y, Tomiyama H, Yoshino H, Asahina M, Kobayshi S, Ng Ar Rosales RL, Hassin-Bear S, Shinar Y, Lu CS, Shimizu N, Toda T, Mizuno Y, Hattori N: Novel PINK1 mutations in early-onset parkinsonism. Ann Neurol 56: 424-427, 2004 Hatano Y, Sato K, Elibol B, Yoshino H, Yamamura Y, Bonifati V, Shinotoh H, Asahina M, Kobayshi S, Ng Ar Rosales RL, Hassin-Bear S, Shinar Y, Lu CS, Chang HC, Wu Chou, YH, Atac FB, Kobayashi T, Toda T, Mizuno Y, Hattori N: PARK6-linked autosomal recessive early-onset parkinsonism in Asian populations. Neurology 63: 1482-1485, 2004 Hattori N, Mizuno Y: Pathogenetic mechanisms of parkin in Parkinson’ s disease. Lancet 364: 722-724, 2004 Higashi Y, Asanuma M, Miyazaki I, Hattori N, Mizuno Y, Ogawa N: Parkin attenuates manganese-induced dopaminergic cell death. J Biol Chem 89: 1490-1497, 2004 Kagohashi M, Okuma Y, Fujishima K, Kitada T, Mizuno Y: Blepharospasm associated with multiple system atrophy: A case report and review of the literature. Parkinsonism Relat Disord 10: 169-171, 2004 Komine-Kobayashi M, Chou N, Mochizuki H, Nakao A, Mizuno Y, Urabe T: Dual role of Fcgamma receptor in transient focal cerebral ischemia in mice. Stroke 35: 958-963, 2004 Motoi Y, Itaya M, Mori H, Mizuno Y, Iwasaki T, Hattori H, Haga S, Ikeda K: Apolipoprotein E receptor 2 is involved in neuritic plaque formation in APP sw mice. Neurosci Lett 368: 144-147, 2004 Motoi Y, Takanashi M, Itaya M, Ikeda K, Mizuno Y, Mori H: Glial localization of four-repeat tau in atypical progressive supranuclear palsy. Neuropathology 24: 60-65, 2004 Shimo-Nakanishi Y, Hasebe T, Suzuki A, Mochizuki H, Npmiyama T, Tanaka Y, Nagaoka I, Mizuno Y, Urabe T: Functional effects of NAD (P) H oxidase p22phox C242T mutation in human leukocytes and association with thrombotic cerebral infarction. Atherosclerosis 175: 109-115, 2004 Tanaka M, Cabrera VM, Gonzalez AM, Larruga JM, Takeyasu T, Fuku N, Guo LJ, Hirose R, Fujita Y, Kurata M, Shinoda K, Umetsu K, Yamada Y, Oshida Y, Sato Y, Hattori N, Mizuno Y, Arai Y, Hirose N, Ohta S, Ogawa O, Tanaka Y, Kawamori R, Shamoto-Nagai M, Maruyama W, Shimokata H, Suzuki R, Shimodaira H: Mitochondrial genome variation in eastern Asia and the peopling of Japan. Genome Res 14: 1832-1850, 2004 Tanaka R, Yamashiro K, Mochizuki H, Cho N, Onodera M, Mizuno Y, Urabe T: Neurogenesis after transient global ischemia in the adult hippocampus visualized by improved retroviral vector. Stroke 35: 1454-1459, 2004 Tsuchiya K, Tajima H, Kuwae T, Takeshima T, Nakano T, Tanaka M, Sunaga K, Fukuhara Y, Nakashima K, Ohama E, Mochizuki H, Mizuno Y, Katsube N, Ishitani R: Pro-apoptotic protein glyceraldehyde-3-phosphate dehydrogenase promotes the formation of Lewy body-like inclusions. Eur J Neurosci 21: 317-326, 2004 Watanabe T, Okuma Y, Fujishima K, Kitada T, Nakao Y, Mizuno Y: Isolated deep venous thrombosis: An important cause for isolated mental impairment. Eur Neurol 52: 123-125, 2004 Yamada M, Iwatubo T, Mizuno Y, Mochizuki H: Overexpression of α-synuclein in rat substantia nigra results in loss of dopaminergic neurons, phosphorylation of α-synuclein and activation of caspase-9: resemblance to pathogenetic changes in Parkinson’s disease. J Neurochem 91: 451-461, 2004 Yamada M, Onodera M, Mizuno Y, Mochizuki H: Neurogenesis in olfactory bulb identified by retroviral labeling in normal and 1-methyl-4-phenyl-1, 2, 3, 6-tetrahydropyridine-treated adult mice. Neuroscience 124: 173-181, 2004 Yamashiro K, Okuma Y, Fujishima K, Goto K, Mizuno Y: Bilateral hypoglossal nerve involvement in chronic inflammatory demyelonating polyneuropathy. Eur Neurol 51: 45-46, 2004 Fukae J, Takanashi M, Kubo S, Nishioka K, Nakabeppu Y, Mori H, Mizuno Y, Hattori N: Expression of 8-oxoguanine DNA glycosylase (OGG1) in Parkinson’s disease and related neurodegenerative disorders. Acta Neuropathol 109: 256-262, 2005 OkumaY, Kagohashi M, Mizuno Y: (Reply to letter to the editor) Neurophysiology of blepharospasm in multiple system strophy. Parkinsonism Relat Disord 11: 203-204, 2005 Okuma Y, Fujishima K, Miwa H, Mori H, Mizuno Y: Myoclonic tremulous movements in multiple system atrophy are a form of cortical myoclonus. Mov Disord 20: 451-456, 2005 Noda K, Kitami T, Gai WP, Chegini F, Jensen PH, Fujimura T, Murayama K, Tanaka K, Mizuno Y, Hattori N: Phosphtorylated IκBα is a component of Lewy body of Parkinson’s disease. Biochem Biophys Res Commun 331: 309-317, 2005 Watanabe T, Okuma Y, Fujishima K, Kitada T, Nakao Y, Mizuno Y: Isolated deep venous thrombosis: An important cause for isolated mental impairment. Eur Neurol 52: 317-326, 2005 Yamada M, Mizuno Y, Mochizuki H: Parkin gene therapy for α-synucleinopathy: A rat model of Parkinson’s disease. Hum Gene Ther 16: 262-270, 2005 Nishioka K, Fujishima K, Kobayashi H, Mizuno Y, Okuma Y: An extremely unusual presentation of varicella zoster viral infection of cranial nerves mimicking Garcin syndrome. Clin Neurol Neurosurg in press 池田　穣衛　(東海大学・総合医学研究所) Matsuyama N, Hadano S, Onoe K, Osuga H, Showguchi-Miyata J, Gondo Y, Ikeda J-E: Identification and characterization of the miniature pig Huntington's disease gene homolog: evidence for conservation and polymorphism in the CAG triplet repeat. Genomics 69: 72-85, 2000 Saitoh Y, Miyamoto N, Okada T, Gondo Y, Showguchi-Miyata J, Hadano S, Ikeda J-E: The RS447 human megasatellite tandem repetitive sequence encodes a novel deubiquitinating enzyme with a functional promotor. Genomics 67: 291-300, 2000 Osuga H, Osuga S, Wang F, Fetni R, Hogan MJ, Slack RS, Hakim AM, Ikeda J-E, Park DS: Cyclin dependent kinases as a therapeutic target for stroke. Proc Natl Acad Sci USA 97: 10254-10259, 2000 Hadano S, Hand CK, Osuga H, Yanagisawa Y, Otomo A, Devon RS, Miyamoto N, Showguchi-Miyata J, Okada Y, Singaraja R, Figlewicz DA, Kwiatkowski T, Hosler B A, Sagie T, Skaug J, Nasir J, Brown RH, Jr Scherer SW, Rouleau GA, Hayden MR, Ikeda J-E: A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nat Genet29: 166-173, 2001 Uchida M, Shimatsu Y, Onoe K, Matsuyama N, Niki R, Ikeda J-E, Imai H: Production of transgenic miniature pigs by pronuclear microinjection.Transgenic Res 10: 577-582, 2001 Hadano S, Yanagisawa Y, Skaug J, Fichter K, Nasir J, Martindale D, Koop BF, Scherer SW, Nicholson DW, Rouleau GA, Ikeda J-E, Hayden MR: Cloning and characterization of three novel genes, ALS2CR1, ALS2CR2, and ALS2CR3, in the juvenile amyotrophic lateral sclerosis (ALS2) critical region at chromosome 2q33-q34: Candidate genes for ALS2. Genomics 71: 200-213, 2001 Wang F, Corbett D, Osuga H, Osuga S, Ikeda J-E, Slack RS, Hogan MJ, Hakim AM, Park DS: Inhibition of cyclin-dependent kinases improves CA1 neuronal survival and behavioral performance after global ischemia in the rat. J Cereb Blood Flow Metab 22: 171-182, 2002 Xu M, Okada T, Sakai H, Miyamoto N, Yanagisawa Y, MacKenzie AE, Hadano S, Ikeda J-E: Functional human NAIP promoter and transcriptional elements of the human NAIP and PsiNAIP genes. Biochim Biophys Acta 1574: 35-50, 2002 Okada T, Gondo Y, Goto J, Kanazawa I, Hadano S, Ikeda J-E: Unstable transmission of the RS447 human megasatellite tandem repetitive sequence that contains the USP17 gene encoding a functional deubiquitinating enzyme. Hum Genet110: 302-313, 2002 Singaraja RR, Hadano S, Metzler M, Givan S, Wellington CL, Warby S, Yanai A, Gutekunst CA, Leavitt BR, Yi H, Fischer K, Gan L, McCutcheon K, Chopra V, Michel J, Hersch SM, Ikeda J-E, Hayden MR: HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. Hum Mol Genet 11: 2815-2828, 2002 Kunita R, Otomo A, Ikeda J-E: Identification and characterization of novel members of the CREG family, putative secreted glycoproteins expressed specifically in brain. Genomics 80: 456-460, 2002 Nagano I, Murakami T, Shiote M, Manabe Y, Hadano S, Yanagisawa Y, Ikeda J-E, Abe K: Single-nucleotide polymorphisms in uncoding regions of ALS2 gene of Japanese patients with autosomal-recessive amyotrophic lateral sclerosis. Neurol Res 25: 505-509, 2003 Otomo A, Hadano S, Okada T, Mizumura H, Kunita R, Nishijima H, Showguchi-Miyata J, Yanagisawa Y, Kohiki E, Suga E, Yasuda M, Osuga H, Nishimoto T, Narumiya S, Ikeda J-E: ALS2, a novel guanine nucleotide exchange factor for the small GTPase Rab5, is implicated in endosomal dynamics. Hum Mol Genet 12: 1671-1687, 2003 Tanaka K, Shouguchi-Miyata J, Miyamoto N, Ikeda J-E: Novel nuclear shuttle proteins, HDBP1 and HDBP2, bind to neuronal cell specific cis-regulatory element in the promoter for the human Huntington’s disease gene. J Biol Chem 279: 7275-7286, 2004 Storbeck CJ, Drmanic S, Daniel K, Waring JD, Jirik FR, Parry DJ, Ahmed N, Sabourin LA, Ikeda J-E, Korneluk RG: Inhibition of myogenesis in transgenic mice expressing the human DMPK 3'-UTR. Mol Genet 13: 589-600, 2004 木村　洋子　(東京都臨床医学総合研究所) Kimura Y, Koitabashi S, Fujita T: Analysis of yeast prion aggregates with amyloid-staining compound in vivo. Cell Struc Func 28: 187-193, 2003 Kimura Y, Koitabashi S, Kakizuka A, Fujita T: The role of pre-exisiting aggregates in Hsp104-dependnent polyglutamine aggregate formation and epigenetic change of yeast prions. Genes Cells 9: 685-696, 2004 青木　正志　(東北大学・医学部附属病院・神経内科) Nagai M, Aoki M, Miyoshi I, Kato M, Pasinelli P, Kasai N, Brown RH Jr, Itoyama Y: Rats expressing human cytosolic Cu/Zn superoxide dismutase transgenes with amyotrophic lateral sclerosis-associated mutations develop motor neuron disease. J Neurosci 21: 9246-9254, 2001 Tateyama M, Aoki M, Nishino I, Hayashi YK, Sekiguchi S, Shiga Y, Takahashi T, Onodera Y, Haginoya K, Kobayashi K, Iinuma K, Nonaka I, Arahata K, Itoyama Y: Mutation in the caveolin-3 gene causes a peculiar form of distal myopathy. Neurology 58: 323-325, 2002 Saito A, Higuchi I, Nakagawa M, Saito M, Hirata K, Suehara M, Yoshida Y, Takahashi T, Aoki M, Osame M: Miyoshi myopathy patients with novel 5' splicing donor site mutations showed different immunostaining at sarcolemma. Acta Neuropathol 104: 615-620, 2002 Nishino I, Noguchi S, Murayama K, Driss A, Sugie K, Oya Y, Nagata T, Chida K, Takahashi T, Takusa Y, Ohi T, Nishimiya J, Sunohara N, Ciafaloni E, Kawai M, Aoki M, Nonaka I: Distal myopathy with rimmed vacuoles is allelic to hereditary inclusion body myopathy. Neurology 59: 1689-1693, 2002 Takahashi T, Aoki M, Tateyama M, Kondo E, Mizuno T, Onodera Y, Takano R, Kawai H, Kamakura K, Mochizuki H, Shizuka-Ikeda M, Nakagawa M, Yoshida Y, Akanuma J, Hoshino K, Saito H, Nishizawa M, Kato S, Saito K, Miyachi T, Yamashita H, Kawai M, Matsumura T, Kuzuhara S, Ibi T, Sahashi K, Nakai H, Kohnosu T, Nonaka I, Arahata K, Brown RHJr, Saito H, Itoyama Y: Dysferlin mutations in Japanese Miyoshi myopathy: Relationship to phenotype. Neurology 60: 1799-1804, 2003 Tobisawa S, Hozumi Y, Arawaka S, Koyama S, Wada M, Nagai M, Aoki M, Itoyama Y, Goto K, Kato T: Mutant SOD1 linked to familial amyotrophic lateral sclerosis, but not wild-type SOD1, induces ER stress in COS7 cells and transgenic mice. Biochem Biophys Res Commun 303: 496-503, 2003 Kato S, Saeki Y, Aoki M, Nagai M, Ishigaki A, Itoyama Y, Kato M, Asayama K, Awaya A, Hirano A, Ohama E: Histological evidence of redox system breakdown caused by superoxide dismutase 1 (SOD1) aggregation is common to mutant SOD-1-linked familial amyotrophic lateral sclerosis patients and transgenic rats expressing human mutant SOD1. Acta Neuropathol 107: 149-58, 2004 Miyazaki K, Fujita T, Ozaki T, Kato C, Kurose Y, Sakamoto M, Kato S, Goto T, Itoyama Y, Aoki M, Nakagawara A: NEDL1, a novel ubiquitin-protein isopeptide ligase for dishevelled-1, targets mutant superoxide dismutase-1. J Biol Chem 279: 11327-11335, 2004 Chang-Hong R, Wada M, Koyama S, Kimura H, Arawaka S, Kawanami T, Kurita K, Kadoya T, Aoki M, Itoyama Y, Kato T: Neuroprotective effect of oxidized galectin-1 in a transgenic mouse model of amyotrophic lateral sclerosis. Exp Neurol 194: 203-211, 2005 青崎　敏彦　(東京都老人総合研究所・神経回路動態研究グループ) Tateno M, Sadakata H, Tanaka M, Itohara S, Shin R-M, Miura M, Masuda M, Aosaki T, Urushitani M, Misawa H, Takahashi R: Calcium-permeable AMPA receptors promote misfolding of mutant SOD1 and development of amyotrophic lateral sclerosis in a transgenic mouse model. Hum Mol Genet 13: 2183-2196, 2004 Shin R-M, Masuda M, Miura M, Sano H, Shirasawa T, Song W-J, Kobayashi K, Aosaki T: Dopamine D4 receptor-induced postsynaptic inhibition of GABAergic currents in mouse globus pallidus neurons. J Neurosci 23: 11662-11672, 2003 Miura M, Watanabe M, Offermanns S, Simon MI, Kano M: Group I metabotropic glutamate receptor signaling via Galpha q/Galpha 11 secures the induction of long-term potentiation in the hippocampal area CA1. J Neurosci 22: 8379-8390, 2002 Nakajima M, Miura M, Aosaki T, Shirasawa T: Deficiency of presenilin-1 increases calcium-dependent vulnerability of neurons to oxidative stress in vitro. J Neurochem 78: 807-814, 2001 Suzuki T, Miura M, Nishimura K, Aosaki T: Dopamine-dependent synaptic plasticity in the striatal cholinergic interneurons. J Neurosci 21: 6492-6501, 2001 荒若　繁樹　(山形大学・医学部) Ren CH, Wada M, Koyama S, Kimura H, Arawaka S, Kawanami T, Kurita K, Kadoya T, Aoki M, Itoyama Y, Kato T: Neuroprotective effect of oxidized galectin-1 in a transgenic mouse model of amyotrophic lateral sclerosis. Exp Neurol 194: 203-211, 2005 有賀　寛芳　(北海道大学・大学院薬学研究科) Taira T, Saito Y, Niki T, Iguchi-Ariga SM, Takahashi K, Ariga H: DJ-1 plays a role in anti-oxidative stress to prevent cell death. EMBO Rep 5: 213-218, 2004 Bandopadhyay R, Kingsbury A, Cookson M, Reid A, Evans I, Hope A, Pittman A, Lashley T, Canet-Aviles R, Miller D, Mc Lendon C, Strand C, Leonard A, Ariga H, Wood N, de Silva R, Hardy J, Holton J, Lees A, Revesz T: The expression of DJ-1 (PARK7) in normal human CNS and idiopathic Parkinson’s disease. Brain 127: 420-430, 2004 Taira T, Iguchi-Ariga SMM, Ariga H: Co-localization with DJ-1 is essential for the androgen receptor to exert its transcription activity that has been impaired by androgen-antagonists. Biol Pharm Bull 27: 574-577, 2004 Kinumi T, Kimata J, Taira T, Ariga H, Niki E: Cysteine-106 of DJ-1 is the most sensitive cysteine residue to hydrogen peroxide mediated oxidation in vivo in human umbilical vein endothelial cells. Biochem Biophys Res Commun 317: 722-728, 2004 Takahashi-Niki K, Niki T, Taira T, Iguchi-Ariga SMM, Ariga H: Reduced anti-oxidative stress activities of DJ-1 mutants found in Parkinson’s disease patients. Biochem Biophys Res Commun 320: 389-397, 2004 Sekito A, Taira T, Niki T, Iguchi-Ariga SM, Ariga H: Stimulation of transforming activity of DJ-1 by Abstrakt, a DJ-1-binding protein. Int J Oncol 26: 685-690, 2005 Shinbo Y, Taira T, Niki T, Iguchi-Ariga SM, Ariga H: DJ-1 restores p53 transcription activity inhibited by Topors/p53BP3. Int J Oncol 26: 641-648, 2005 Shinbo Y, Niki T, Taira T, Ooe H, Takahashi-Niki K, Maita C, Seino C, Iguchi-Ariga SMM, Ariga H: Proper SUMO-1 conjugation is essential to DJ-1 to exert its full activities. Cell Death Differ in press 五十嵐　修一　(新潟大学・脳研究所) Sano Y, Date H, Igarashi S, Onodera O, Oyake M, Takahashi T, Hayashi S, Morimatsu M, Takahashi H, Makifuchi T, Fukuhara N, Tsuji S: Aprataxin, the causative protein for early-onset ataxia with ocular motor apraxia and hypoalbuminemia, is a nuclear protein with a potential role as a DNA repair protein.Ann Neurol 55: 241-249, 2004 Date H, Igarashi S, Sano Y, Takahashi T, Takahashi T, Takano H, Tsuji S, Nishizawa M, Onodera O: The FHA domain of aprataxin interacts with the C-terminal region of XRCC1. Biochem Biophys Res Commun 325: 1279-1285, 2004 池谷　裕二　(東京大学・薬学部) Baba A, Yamada MK, Nishiyama N, Matsuki N, Ikegaya Y: Different Ca2+ dynamics between isolated hippocampal pyramidal cells and dentate granule cells. J Neurocytol 31: 41-48, 2002 Nakao K, Ikegaya Y, Yamada MK, Nishiyama N, Matsuki N: Hippocampal long-term depression as an index of spatial working memory. Eur J Neurosci 16: 970-914, 2002 Ikegaya Y, Matsuura S, Ueno S, Baba A, Yamada MK, Nishiyama N, Matsuki N: βAmyloid enhances glial glutamate uptake activity and attenuates synaptic efficacy. J Biol Chem 277: 32180-32186, 2002 Yamada MK, Nakanishi K, Ohba S, Nakamura T, Ikegaya Y, Nishiyama N, Matsuki N: Brain-derived neurotrophic factor promotes the maturation of GABAergic mechanisms in cultured hippocampal neurons. J Neurosci 22: 7580-7585, 2002 Ikegaya Y, Ishizaka Y, Matsuki N: BDNF attenuates hippocampal LTD via activation of phospholipase C: Implications for a vertical shift in the frequency-response curve of synaptic plasticity. Eur J Neurosci 16: 145-148, 2002 Ikegaya Y, Matsuki N: Vasopressin induces emesis in Suncus murinus. Jpn J Pharmacol 89: 324-326, 2002 Kim J-A, Mitsukawa K, Yamada MK, Nishiyama N, Matsuki N, Ikegaya Y: Cytoskeleton disruption causes apoptotic degeneration of dentate granule cells in hippocampal slice cultures. Neuropharmacology 42: 1109-1118, 2002 Ueno S, Tsukamoto M, Hirano T, Kikuchi K, Yamada MK, Nishiyama N, Nagano T, Matsuki N, Ikegaya Y: Mossy fiber Zn2+ spillover modulates heterosynaptic N-methyl-D-aspartate receptor activity in hippocampal CA3 circuits. J Cell Biol 158: 215-220, 2002 Ikegaya Y, Matsuki N: Regionally selective neurotoxicity of NMDA and colchicine is independent of hippocampal neural circuitry. Neuroscience 113: 253-256, 2002 Ikegaya Y, Koyama R,Yamada MK, Nishiyama N, Matsuki N: Rapid regrowth of hippocampal mossy fibres and preceding maturation of NMDA receptor-mediated neurotransmission. Eur J Neurosci 15: 1859-1862, 2002 Iwahori Y, Ikegaya Y, Matsuki N: Hyperpolarization-activated current I(h) in nucleus of solitary tract neurons: Regional difference in serotonergic modulation. Jpn J Pharmacol 88: 459-462, 2002 Koyama R, Yamada MK, Nishiyama N, Matsuki N, Ikegaya Y: Group II metabotropic glutamate receptor activation is required for normal hippocampal mossy fibre development in the rat. J Physiol 539: 157-162, 2002 Matsuura S, Ikegaya Y, Yamada MK, Nishiyama N, Matsuki N: Endothelin downregulates the glutamate transporter GLAST in cAMP-differentiated astrocytes in vitro. Glia 37: 178-182, 2002 Baba A, Mitsumori K, Yamada MK, Nishiyama N, Matsuki N, Ikegaya Y: β-Amyloid prevents excitotoxicity via recruitment of glial glutamate transporters. Naunyn Schmiedebergs Arch Pharmacol 368: 234-238, 2003 Baba A, Yasui T, Fujisawa S, Yamada RX, Yamada MK, Nishiyama N, Matsuki N, Ikegaya Y: Activity-evoked capacitative Ca2+ entry: Implications in synaptic plasticity. J Neurosci 23: 7737-7741, 2003 Kim J-A, Yamada MK, Nishiyama N, Matsuki N, Ikegaya Y: Mossy fiber pathfinding in multilayer organotypic cultures of rat hippocampal slices. Cell Mol Neurobiol 23: 115-119, 2003 Tsukamoto M, Yasui T, Yamada MK, Nishiyama N, Matsuki N, Ikegaya Y: Mossy fibre synaptic NMDA receptors trigger non-Hebbian long-term potentiation at entorhino-CA3 synapses in the rat. J Physiol 546: 665-675, 2003 Nakao K, Ikegaya Y, Yamada MK, Nishiyama N, Matsuki N: Fimbrial control of bidirectional synaptic plasticity of medial perforant path-dentate transmission. Synapse 47: 163-168, 2003 Ikegaya Y, Delcroix I Iwakura Y, Matsuki N, Nishiyama N: Interleukin-1α abrogates long-term depression of hippocampal CA1 synaptic transmission. Synapse 47: 54-57, 2003 石川　欽也　(東京医科歯科大学・医学部附属病院・神経内科) Ishikawa K, Nagaoka U, Takashima M, Yoshizawa K, Yoshizawa T, Ishikawa M, Yamawaki T, Shoji S, Mizusawa H: A gene on SCA4 locus causes dominantly inherited pure cerebellar ataxia. Neurology 54: 1971-1975, 2000 Takashima M, Ishikawa K, Nagaoka U, Shoji S, Mizusawa H: A linkage disequilibrium at the candidate gene locus for the 16q-linked autosomal dominant cerebellar ataxia type III in Japan. J Hum Genet 46: 167-171, 2001 Ishikawa K, Owada K, Ishida K, Fujigasaki H, Shun Li M, Tsunemi T, Ohkoshi N, Toru S, Mizutani T, Hayashi M, Arai N, Hasegawa K, Kawanami T, Kato T, Makifuchi T, Shoji S, Tanabe T, Mizusawa H: Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells. Neurology 56: 1753-1756, 2001 Tsunemi T, Saegusa H, Ishikawa K, Nagayama S, Murakoshi T, Mizusawa H, Tanabe T: Novel Cav2.1 splice variants isolated from Purkinje cells do not generate P-type Ca2+ current. J Biol Chem 277: 7214-7221, 2002 Tomimitsu H, Shimizu J, Ishikawa K, Ohkoshi N, Kanazawa I, Mizusawa H: Distal myopathy with rimmed vacuoles: novel mutations in the GNE gene. Neurology 59: 451-454, 2002 Li M, Ishikawa K, Toru S, Tomimitsu H, Takashima M, Goto J, Takiyama Y, Sasaki H, Imoto I, Inazawa J, Toda T, Kanazawa I, Mizusawa H: Physical map and haplotype analysis of 16q-linked autosomal dominant cerebellar ataxia (ADCA) type III in Japan. J Hum Genet 48: 111-118, 2003 Kubodera T, Yokota T, Ohwada K, Ishikawa K, Miura H, Matsuoka T, Mizusawa H: Proteolytic cleavage and cellular toxicity of the human alpha1A calcium channel in spinocerebellar ataxia type 6. Neurosci Lett 341: 74-78, 2003 Yashima T, Noguchi Y, Ishikawa K, Mizusawa H, Kitamura K: Mutation of the EYA1 gene in patients with branchio-oto syndrome. Acta Otolaryngol 123: 279-282, 2003 Takahashi H, Ishikawa K, Tsutsumi T, Fujigasaki H, Kawata A, Okiyama R, Fujita T, Yoshizawa K, Yamaguchi S, Tomiyasu H, Yoshii F, Mitani K, Shimizu N, Yamazaki M, Miyamoto T, Orimo T, Shoji S, Kitamura K, Mizusawa H: Clinical and genetic study in a large cohort of patients with spinocerebellar ataxia type 6. J Hum Genet 49: 256-264, 2004 Tomimitsu H, Shimizu J, Ishikawa K, Ohkoshi N, Kanazawa I, Mizusawa H: Distal myopathy with rimmed vacuoles (DMRV): new GNE mutations and splice variant. Neurology 62: 1607-1610, 2004 Owada K, Ishikawa K, Toru S, Ishida G, Gomyoda M, Tao O, Noguchi Y, Kitamura K, Kondo I, Noguchi E, Arinami T, Mizusawa H: A clinical, genetic, and neuropathologic study in a family with 16q-linked ADCA type III. Neurology 65: 629-632, 2005 Ishikawa K, Toru S, Tsunemi T, Li M, Kobayashi K, Yokota T, Amino T, Owada K, Fujigasaki H, Sakamoto M, Tomimitsu H, Takashima M, Kumagai J, Noguchi Y, Kawashima Y, Ohkoshi N, Ishida G, Gomyoda M, Yoshida M, Hashizume Y, Saito Y, Murayama S, Yamanouchi H, Mizutani T, Kondo I, Toda T, Mizusawa H: An autosomal dominant cerebellar ataxia linked to chromosome 16q22.1 is associated with a single nucleotide substitution in the 5’-untranslated region of the gene encoding a protein with spectrin and Rho guanine-nucleotide exchange factor domains. Am J Hum Genet 77: 280-296, 2005 石黒　啓司　(藤田保健衛生大学・総合医科学研究所) Nakano N, Higashiyama S, Kajihara K, Endo T, Ishiguro H, Yamada K, Nagatsu T, Taniguchi N: NTAKalpha and beta isoforms stimulate brest tumor cell growth by means of different receptor combinations. J Biochem 127: 925-930, 2000 Yamada K, Ichino N, Nishii K, Sawada H, Higashiyama S, Ishiguro H, Nagatsu T: Characterizationof the human NTAK gene structure and distribution if the isoform for rat NTAK mRNA. Gene 255: 15-24, 2000 Shimohata T, Nakajima T, Yamada M, Uchida C, Onodera O, Naruse S, Kimura T, Koide R, Nozaki K, Sano Y, Ishiguro H, Sakoe K, Ooshima T, Sato A, Ikeuchi T, Oyake M, Sato T, Aoyagi Y, Hozumi I, Nagatsu T, Takiyama Y, Nishizawa M, Goto J, Kanazawa I, Davidson I, Tanese N, Takahashi H, Tsuji S: Expanded polyglutamine stretches associated with CAG repeat diseases interact with TAFII130, interfering with CREB-dependent transcription. Nat Genet 26: 29-35, 2000 Nagoya Y, Ishiguro H, Nukina N: DMSO and glycerol reduce bacterial death induced by expressioinof truncated N-terminal huntingtin with expanded polyglutamine tracts. Biochim Biophys Acta 1502: 247-256, 2000 Tokita Y, Keino H, Matsui F, Aono S, Ishiguro H, Higashiyama S, Oohira A: Regulation of neuregulin expression in the injured rat brain and cultured astrocytes. J Neurosci 21: 1257-1264, 2001 Ishiguro H, Yamada K, Sawada H, Nishii K, Ichino N, Sawada M, Kurosawa Y, Matsushita N, Kobayashi K, Goto J, Hashida H, Masuda N, Kanazawa I, Nagatsu T: Age-dependent and tissue-specific CAG repeat instability occurs in mouse knock-in for a mutant Huntington's disease gene. J Neurosci Res 65: 289-297, 2001 一瀬　宏　(藤田保健衛生大学・総合医科学研究所) Mogi M, Togari A, Kondo T, Mizuno Y, Komure O, Kuno S, Ichinose H, Nagatsu T: Caspase activities and tumor necrosis factor receptor R1 (p55) level are elevated in the substantia nigra from Parkinsonian brain. J Neural Transm 107: 335-341, 2000 Hibiya M, Ichinose H, Ozaki N, Fujita K, Nishimoto T, Yoshikawa T, Asano Y, Nagatsu T: Normal values and age-dependent changes in GTP cyclohydrolase I activity in stimulated mononuclear blood cells measured by high-performance liquid chromotography. J Chromatogr B 740: 35-42, 2000 Shen Y, Muramatsu SI, Ikeguchi K, Fujimoto KI, Fan DS, Ogawa M, Mizukami H, Urabe M, Kume A, Nagatsu I, Urano F, Suzuki T, Ichinose H, Nagatsu T, Monahan J, Nakano I, Ozawa K: Triple transduction with adeno-associated virus vectors expressing tyrosine hydroxylase, aromatic L-amino acid decarboxylase, and GTP cyclohydrolase I for gene therapy of Parkinson's disease. Hum Gene Ther 11: 1509-1519, 2000 Mogi M, Togari A, Tanaka K, Ogawa N, Ichinose H, Nagatsu T: Increase in level of tumor necrosis factor-α in 6-hydroxydopamine-lesioned striatum in rats is suppressed by immunosuppressant FK506. Neurosci Lett 289: 165-168, 2000 Tazawa M, Ohtsuki M, Sumi-Ichinose C, Shiraishi H, Kuroda R, Hagino Y, Nakashima S, Nozawa Y, Ichinose H, Nagatsu T, Nomura T: GTP cyclohydrolase I from Tetrahymena pyriformis: cloning of cDNA and expression. Comp Biochem Physiol B 127: 65-73, 2000 Ichinose H, Inagaki H, Suzuki T, Ohye T, Nagatsu T: Molecular mechanisms of hereditary progressive dystonia with marked diurnal fluctuation, Segawa's disease. Brain Dev 22: S107-S110, 2000 Kaneko S, Hikida T, Watanabe D, Ichinose H, Nagatsu T, Kreitman RJ, Pastan I, Nakanishi S: Synaptic integration mediated by striatal cholinergic interneurons in basal ganglia function. Science 289: 633-637, 2000 Maeda T, Haeno S, Oda K, Mori D, Ichinose H, Nagatsu T, Suzuki T: Studies on the genotype-phenotype relation in the hph-1 mouse mutant deficient in guanosine triphosphate (GTP) cyclohydrolase I activity. Brain Dev 22: S50-S53, 2000 Zabetian CP, Anderson GM, Buxbaum SG, Elston RC, Ichinose H, Nagatsu T, Kim KS, Kim CH, Malison RT, Gelernter J, Cubells JF: A quantitative-trait analysis of human plasma-dopamine β-hydroxylase activity: Evidence for a major functional polymorphism at the DBH locus. Am J Hum Genet 68: 515-522, 2001 Sugimoto T, Ikemoto K, Murata S, Tazawa M, Nomura T, Hagino Y, Ichinose H, Nagatsu T, Wada A: A convenient determination of chiral pteridines: Application of fluorescence detected circular dichrolism (FDCD) to the major pterin from Escherichia coli. Heterocycles 54: 283-290, 2001 Mogi M, Togari A, Kondo T, Mizuno Y, Kogure O, Kuno S, Ichinose H, Nagatsu T: Glial cell line-derived neurotrophic factor in the substantia nigra from control and parkinsonian brains. Neurosci Lett 300: 179-181, 2001 Sugimoto T, Ikemoto K, Murata S, Tazawa M, Nomura T, Hagino Y, Ichinose H, Nagatsu T: Identification of (6R)-5,6,7,8-tetrahydro-D-monopterin (=(6R)-2-amino-5,6,7,8-tetrahydro-6-[(1R,2R)-1,2,3-trihydroxypropyl]-pteridin-4(3H)-one) as the native pteridine in Tetrahymena pyriformis. Helv Chim Acta 84: 918-927, 2001 Sumi-Ichinose C, Urano F, Kuroda R, Ohye T, Kojima M, Tazawa M, Shiraishi H, Hagino Y, Nagatsu T, Nomura T, Ichinose H: Catecholamines and serotonin are differently regulated by tetrahydrobiopterin: a study from 6-pyruvoyltetrahydropterin synthase knockout mice. J Biol Chem 276: 41150-41160, 2001 Ohye T, Ichinose H, Yoshizawa T, Kanazawa I, Nagatsu T: A new splicing variant for human tyrosine hydroxylase in the adrenal medulla. Neurosci Lett 312: 157-160, 2001 Kawasaki H, Suemori H, Mizuseki K, Watanabe K, Urano F, Ichinose H, Haruta M, Takahashi M, Yoshikawa K, Nishikawa S, Nakatsuji N, Sasai Y: Generation of TH+ dopaminergic neurons and Pax6+ pigmented epithelia from primate ES cells by SDIA. Proc Natl Acad Sci USA 99: 1580-1585, 2002 Chen N, Ikemoto K, Sugimoto T, Murata S, Ichinose H, Nagatsu T: A highly sensitive and specific FDCD method for chirality analysis of naturally occuring pteridines. Heterocycles 56: 387-392, 2002 Ikemoto K, Sugimoto T, Murata S, Tazawa M, Nomura T, Ichinose H, Nagatsu T: (6R)-5,6,7,8-tetrahydro-L-monapterin from Escherichia coli, a novel natural unconjugated tetrahdropterin. Biol Chem 383: 325-330, 2002 Muramatsu S, Fujimoto K, Ikeguchi K, Shizuma N, Kawasaki K, Ono F, Shen Y, Wang L, Mizukami H, Kume A, Matsumura M, Nagatsu I, Urano F, Ichinose H, Nagatsu T, Terao K, Nakano I, Ozawa K: Behavioral recovery in a primate model of Parkinson's disease by triple transduction of striatal cells with adeno-associated viral vectors expressing dopamine-synthesizing enzymes. Hum Gene Ther 13: 345-354, 2002 Wang L, Muramatsu S, Lu Y, Ikeguchi K, Fujimoto K, Okada T, Mizukami H, Hanazono Y, Kume A, Urano F, Ichinose H, Nagatsu T, Nakano I, Ozawa K: Delayed delivery of AAV-GDNF prevents nigral neurodegeneration and promotes functional recovery in a rat model of Parkinson's disease. Gene Ther 9: 381-389, 2002 Ohtsuki M, Shiraishi H, Kato T, Kuroda R, Tazawa M, Sumi-Ichinose C, Tada S, Udagawa Y, Itoh M, Hishida H, Ichinose H, Nagatsu T, Hagino Y, Nomura T: cAMP inhibits cytokine-induced biosynthesis of tetrahydrobiopterin in human umbilical vein endothelial cells. Life Sci 70: 2187-2198, 2002 Suzuki T, Inagaki H, Yamakuni T, Nagatsu T, Ichinose H: Enhanced expression of GTP cyclohydrolase I in V-1-overexpressing PC12D cells. Biochem Biophys Res Commun 293: 962-968, 2002 Suzuki T, Yamakuni T, Hagiwara M, Ichinose H: Identification of ATF-2 as a transcriptional regulator for the tyrosine hydroxylase gene. J Biol Chem 277: 40768-40774, 2002 Ikemoto K, Suzuki T, Ichinose H, Ohye T, Nishimura A, Nishi K, Nagatsu I, Nagatsu T: Localization of sepiapterin reductase in the human brain. Brain Res 954: 237-246, 2002 Nielsen AL, Sanchez C, Ichinose H, Cervino M, Lerouge T, Chambon P, Losson R: Selective interaction between the chromatin-remodeling factor BRG1 and the heterochromatin-associated protein HP1alpha. EMBO J 21: 5797-5806, 2002 Lee MA, Lee HS, Lee HS, Cho KG, Jin BK, Sohn S, Lee YS, Ichinose H, Kim SU: Overexpression of midbrain-specific transcription factor Nurr1 modifies susceptibility of mouse neural stem cells to neurotoxins. Neurosci Lett 333: 74-78, 2002 Ihara M, Kohara N, Urano F, Ichinose H, Takao S, Nishida T, Saiki H, Kawamoto Y, Ikeda A, Takagi S, Shibasaki H: Neuroleptic malignant syndrome with prolonged catatonia in a dopa-responsive dystonia patient. Neurology 59: 1102-1104, 2002 Ihara M, Kohara N, Urano F, Ichinose H, Takao S, Nishida T, Saiki H, Kawamoto Y, Ikeda A, Takagi S, Shibasaki H: Generation of human artificial chromosomes expressing naturally controlled guanosine triphosphate cyclohydrolase I gene. Genes Cells 7: 1021-1032, 2002 大野　耕策　(鳥取大学・医学部付属病院・脳神経小児科) Sawamura N, Gong JS, Garver WS, Heidenreich RA, Ninomiya H, Ohno K, Yanagisawa K, Michikawa M: Site-specific phosphorylation of tau accompanied by activation of mitogen-activated protein kinase (MAPK) in brains of Niemann-Pick type C mice. J Biol Chem 276: 10314-10319, 2001 Higaki K, Ninomiya H, Sugimoto Y, Suzuki T, Taniguchi M, Niwa H, Pentchev PG, Vanier MT, Ohno K: Isolation of NPC1 deficient Chinese hamster ovary cell mutants by gene trap mutagenesis. J Biochem 129: 875-880, 2001 Yamada A, Saji M, Ukita Y, Shinoda Y, Taniguchi M, Higaki K, Ninomiya H, Ohno K: Progressive cell loss in the ventral posterior lateral and medial nuclei of thalamus in Niemann-Pick disease type C mice brain. Brain Dev 23: 288-297, 2001 Millat G, Marcais C, Tomasetto C, Chikh K, Fensom AH, Harzer K, Wenger DA, Ohno K, Vanier MT: Niemann-Pick C1 disease: correlations between NPC1 mutations, levels of NPC1 protein and phenotypes emphasize the functional significance of the putative sterol-sensing domain and of the cysteine-rich luminal loop. Am J Hum Genet 68:1373-1385, 2001 Watabe K, Ida H, Uehara K, Oyanagi K, Sakamoto T, Tanaka J, Garver WS, Miyawaki S, Ohno K, Eto Y: Establishment and characterization of immortalized Schwann cells from murine model of Niemann-Pick disease type C (spm/spm). J Peripher Nerv Syst 6: 85-94, 2001 Taniguchi M, Shinoda Y, Ninomiya H, Vanier MT, Ohno K: Sites and temporal changes of gangliosides GM1/GM2 storage in the Niemann-Pick disease type C mouse brain. Brain Dev 23: 414-421, 2001 Sugimoto Y, Ninomiya H, Ohsaki Y, Higaki K, Davies JP, Ioannou YA, Ohno K: Accumulation of cholera toxin and GM1 ganglioside in the early endosome of Niemann-Pick C1-deficient cells. Proc Natl Acad Sci USA 98: 12391-12396, 2001 Saito Y, Suzuki K, Nanba E, Yamamoto T, Ohno K, Murayama S: Niemann-Pick type C disease: accelerated neurofibrillary tangle formation and amyloid β deposition associated with APOE ε4 hmomozygosity. Ann Neurol 52: 351-355, 2002 Ohsaki Y, Sugimoto Y, Suzuki M, Kaidoh T, Shimada Y, Ohno-Iwashita Y, Davies JP, Ioannou YA, Ohno K, Ninomiya H: Reduced sensitivity of Niemann-Pick C1-deficient cells to theta-toxin (perfringolysin O): sequestration of toxin to raft-enriched membrane vesicles. Histochem Cell Biol 121: 263-272, 2004 Yamamoto T, Feng JH, Higaki K, Taniguchi M, Nanba E, Ninomiya H, Ohno K: Increased NPC1 mRNA in skin fibroblasts from Niemann-Pick disease type C patients. Brain Dev 26: 245-250, 2004 Ohara S, Ukita Y, Ninomiya H, Ohno K: Axonal dystrophy of dorsal root ganglion sensory neurons in a mouse model of Niemann-Pick disease type C. Exp Neurol 187: 289-298, 2004 郭　伸　(東京大学・医学部附属病院・神経内科) Yamamoto Y, Hidaka I, Nozaki D, Iso-o N, Soma R, Kwak S: Noise-induced sensitization of human brain. Physica A 314: 53-60, 2002 Yoshimura M, Yamamoto T, Iso-o N, Imafuku I, Momose T, Shirouzu I, Kwak S, Kanazawa I: Hemiparkinsonism in a patient with a mesencephalic tumor. J Neurol Sci 197: 89-92, 2002 Yamamoto Y, Hidaka I, Iso-o N, Komai A, Soma R, Kwak S: Noise-based compensation for postural hypotension in primary autonomic failure. Brain Res 945: 71-78, 2002 Kawahara Y, Kwak S, Sun H, Ito K, Hashida H, Aizawa H, Jeong SY, Kanazawa I: Human spinal motoneurons express low relative abundance of GluR2 mRNA: An implication for excitotoxicity in ALS. J Neurochem 85: 680-689, 2003 Kawahara Y, Ito K, Sun H, Kanazawa I, Kwak S: Low editing efficiency of GluR2 mRNA is associated with a low relative abundance of ADAR2 mRNA in white matter of normal human brain. Eur J Neurosci 18: 23-33, 2003 Soma R, Nozaki D, Kwak S, Yamamoto Y: 1/f noise outperforms white noise in sensitizing baroreflex function in the human brain. Phys Rev Lett 91: 078101-1-4, 2003 KawaharaY, ItoK, SunH, ItoM, Kanazawa I, Kwak S: Regulation of glutamate receptor RNA editing and ADAR mRNA expression in developing human normal and Down’s syndrome brains. Dev Brain Res 148: 151-155, 2004 Kawahara Y, Ito K, Sun H, Aizawa H, Kanazawa I, Kwak S: RNA editing and death of motor neurons. Nature 427: 801, 2004 Kawahara Y, Ito K, Sun H, Ito M, Kanazawa I, Kwak S: GluR4c, an alternative splicing isoform of GluR4 is abundantly expressed in adult human brain. Mol Brain Res 127: 150-155, 2004 Struzik ZR, Hayano J, Sakata S, Kwak S, Yamamoto Y: 1/f scaling in heart rate requires antagonistic autonomic control. Phys Rev E 70: 050901(R)-1-4, 2004 Sun H, Kawahara Y, Ito K, Kanazawa I, Kwak S: Expression profile of AMPA receptor subunit mRNA in single adult rat brain and spinal cord neurons in situ. Neurosci Res 52: 228-234, 2005 Kwak S, Kawahara Y: Deficient RNA editing of GluR2 and neuronal death in ALS. J Mol Med 83: 110-120, 2005 Yamamoto Y, Struzik ZR, Soma R, Ohashi K, Kwak S: Noisy vestibular stimulation improves autonomic and motor responsiveness in central neurodegenerative disorders. Ann Neurol 58: 175-181, 2005 嘉村　巧　(九州大学・生体防御医学研究所) Kamura T, Hara T, Kotoshiba S, Yada M, Ishida N, Imaki H, Hatakeyama S, Nakayama K, Nakayama KI: Degradation of p57Kip2 mediated by SCFSkp2-dependent ubiquitylation. Proc Natl Acad Sci USA 100: 10231-10236, 2003 Imaki H, Nakayama K, Delehouzee S, Handa H, Kitagawa M, Kamura T, Nakayama KI: Cell cycle-dependent regulation of the Skp2 promoter by GA-binding protein. Cancer Res 63: 4606-4613, 2003 Oshikawa K, Matsumoto M, Yada M, Kamura T, Hatakeyama S, Nakayama KI: Preferential interaction of TIP120A with Cul1 that is not modified by NEDD8 and not associated with Skp1. Biochem Biophys Res Commun 303: 1209-1216, 2003 Ishida N, Hara T, Kamura T, Yoshida M, Nakayama K, Nakayama KI: Cytoplasmic ubiquitin ligase KPC regulates proteolysis of p27Kip1 at G1 phase. J Biol Chem 6: 1229-1235, 2004 Kamura T, Maenaka K, Kotoshiba S, Matsumoto M, Kohda D, Conaway RC, Conaway JW, Nakayama KI: VHL-box and SOCS-box domains determine binding specificity for Cul2-Rbx1 and Cul5-Rbx2 modules of ubiquitin ligases. Genes Dev 18: 3055-3065, 2004 Okumura F, Hatakeyama S, Matsumoto M, Kamura T, Nakayama KI: Functional regulation of FEZ1 by the U-box-type ubiquitin ligase E4B contributes to neuritogenesis. J Biol Chem 279: 53533-53543, 2004 Tamamori-Adachi M, Hayashida K, Nobori K, Omizu C, Yamada K, Sakamoto N, Kamura T, Fukuda K, Ogawa S, Nakayama KI, Kitajima S: Down-regulation of p27Kip1 promotes cell proliferation of rat neonatal cardiomyocytes induced by nuclear expression of cyclin D1 and CDK4: Evidence for impaired Skp2-dependent degradation of p27 in terminal differentiation. J Biol Chem 279: 50429-50436, 2004 Yan Q, Kamura T, Cai Y, Jin J, Ivan M, Mushegian A, Conaway RC, Conaway JW: Identification of elongin C and Skp1 sequences that determine cullin selection. J Biol Chem 279: 43019-43026, 2004 Yada M, Hatakeyama S, Kamura T, Nishiyama M, Tsunematsu R, Imaki H, Ishida N, Okumura F, Nakayama K, Nakayama KI: Phosphorylation- dependent degradation of c-Myc is mediated by the F-box protein Fbw7. EMBO J 23: 2116-2125, 2004 Hatakeyama S, Matsumoto M, Kamura T, Murayama M, Chui DH, Planel E, Takahashi R, Nakayama KI, Takashima A: U-box protein carboxyl terminus of Hsc70-interacting protein (CHIP) mediates poly-ubiquitylation preferentially on four-repeat Tau and is involved in neurodegeneration of tauopathy. J Neurochem 91: 299-307, 2004 Kotoshiba S, Kamura T, Hara T, Ishida N, Nakayama KI: Molecular dissection of the interaction between p27 and KPC, the ubiquitin ligase that regulates proteolysis of p27 in G1 phase. J Biol Chem 280: 17694-17700, 2005 亀高　諭　(大阪大学・大学院医学系研究科) Koike M, Shibata M, Ohsawa Y, Nakanishi H, Koga T, Kametaka S, Waguri S, Momoi T, Kominami E, Peters C, Figura K, Saftig P, Uchiyama Y: Involvement of two different cell death pathways in retinal atrophy of cathepsin D-deficient mice. Mol Cell Neurosci 22: 146-161, 2003 Koike M, Shibata M, Ohsawa Y, Kametaka S, Waguri S, Kominami E, Uchiyama Y: The expression of tripeptidyl peptidase I in various tissues of rats and mice. Arch Histol Cytol 65: 219-232, 2002 河田　康志　(鳥取大学・工学部・生物応用工学科) Hayashi K, Ying L, Singh S, Kaneko S, Nirasawa S, Shimonishi T, Kawata Y, Imoto T, Kitaoka M: Improving enzyme characteristics by gene shuffling: Application to β-glucosidase. J Mol Catalysis B, Enzymatic 11: 811-816, 2001 Higurashi T, Ichimura K, Mizobata T, Nagai J, Kawata Y, Hiragi Y：Unfolded structure of Escherichia coli co-chaperonin GroES. Photon Factory Activity Rep 18: 236, 2001 Inoue K, Miyazaki T, Mizobata T, Kawata Y, Yagi N: Studies on the function of chaperonin GroEL. SPring-8 User Exp Rep 7: 160, 2001 Yagi H, Miyazaki T, Ikeda M, Higurashi T, Inoue K, Mizobata T, Kawata Y.: Conformationa change of amyloid fibril formation-competent proteins at various solution environments by small-angle X-ray scattering. SPring-8 User Exp Rep 7: 165, 2001 Yanase H, Moriya K, Mukai N, Kawata Y, Okamoto K, Kato N: Effects of GroESL coexpression on the folding of nicotinoprotein formaldehyde dismutase from Pseudomonas putida F61. Biosci Biotechnol Biochem 66: 85-91, 2002 Nakayama N, Matsubara T, Ohshiro T, Moroto Y, Kawata Y, Koizumi K, Hirakawa Y, Suzuki M, Maruhashi K, Izumi Y, Kurane R: A novel enzyme, 2'-hydroxybiphenyl-2-sulfinate desulfinase(DszB), from a dibenzothiophene-desulfurizing bacterium Rhodococcus erythropolis KA2-5-1: gene overexpression and enzyme characterization. Biochim Biophys Acta 1598: 122-130, 2002 Miyazaki T, Yoshimi T, Furutsu Y, Hongo K, Mizobata T, Kanemori M, Kawata Y: GroEL-substrate-GroES ternary complexes are an important transient intermediate of the chaperonin cycle. J Biol Chem 277: 50621-50628, 2002 Fujii T, Sakai H, Kawata Y, Hata Y: Crystal structure of thermostable aspartase and exploration of functional site in aspartase family. Acta Crystallogr A58, C100, 2002 Yagi H, Miyazaki T, Ikeda M, Inoue K, Hongo K, Mizobata T, Kawata Y.: Analysis of amyloid fibril formation-competent proteins at various solution environments by small-angle X-ray scattering: a case of α-synuclein at 1.0 M NaCl. SPring-8 User Exp Rep 8: 166, 2002 Ikeda M, Yagi H, Miyazaki T, Inoue K, Mizobata T, Kawata Y.: Conformational change detection of amyloid fibril formation-competent protein GroES at various slution environments by small-angle X-ray scattering. SPring-8 User Exp Rep 8: 183, 2002 喜多　淑子　(慶應義塾大学・医学部) Chiba T, Hashimoto Y, Tajima H, Yamada M, Kato R, Niikura T, Terashita K, Schulman H, Aiso S, Kita Y, Matsuoka M, Nishimoto I: Neuroprotective effect of activity-dependent neurotrophic factor, ADNF, agaist toxicity by familial amyotrophic lateral sclerosis-linked mutant SOD1 in vitro and in vivo. J Neurosci Res 78: 542-552, 2004 Hashimoto Y, Kaneko Y, Tsukamoto E, Frankowski H, Kouyama K, Kita Y, Niikura T, Aiso S, Bredesen DE, Matsuoka M, Nishimoto I: Molecular characterization of neurohybrid cell death induced by Alzheimer’s amyloid-β peptides via p75NTR/PLAIDD. J Neurochem 90: 549-558, 2004 Hashimoto Y, Terashita K, Niikura T, Yamagishi Y, Ishizaka M, Kanekura K, Chiba T, Yamada M, Kita Y, Aiso S, Matsuoka M, Nishimoto I: Humanin antagonists: dimerizaiton-interfering mutants inhibit neuroprotection of Humanin.  Eur J Neurosci 19: 2356-2364,2004 Obata Y, Niikura T, Kanekura K, Hashimoto Y, Kawasumi M, Kita Y, Aiso S, Matsuoka M, Nishimoto I: Expression of N19S-SOD1, an SOD1 mutant found in sporadic amyotrophic lateral sclerosis patients, induces low-grade motoneuronal toxicity. J Neurosci Res 81: 720-729, 2005 Yamada M, Chiba T, Sasabe J, Nawa M, Tajima H, Niikura T, Terashita K, Aiso S, Kita Y, Matsuoka M, Nishimoto I: Implanted cannula-mediated repetitive administration of amyloid beta into the mouse cerebral venticle effectively impairs spatial working memory. Behav Brain Res 164: 139-146, 2005 Nagayoshi C, Ishibashi M, Kita Y, Matsuoka M, Nishimoto I, Tokunaga M: Expression, refolding and characterization of human brain serine racemase in Escherichia Coli with N-terminal HIS-tag. Protein Pept Lett 12: 487-490, 2005 Kanekura K, Hashimoto Y, Kita Y, Sasabe J, Aiso S, Nishimoto I, Matsuoka M: A Rac1/phosphatidylinositol-3 kinase/Akt3 anti-apoptotic pathway, triggered by AlsinLF, the product of ALS2 gene, antagonized Cu/Zn-superoxide dismutase. J Biol Chem 280: 4532-4543, 2005 Tajima H, Kawasumi M, Chiba T, Yamada M, Yamashita K, Nawa M, Kita Y, Kouyama K, Aiso S, Matsuoka M, Niikura T, Nishimoto I: A Humanin derivative, S14G-HN, prevents amyloid-β-induced memory impairment in mice. J Neurosci Res 79: 714-723, 2005 木山　博資　(大阪市立大学・大学院医学研究科) Namikawa K, Honma M, Abe K, Takeda M, Mansur K, Obata T, Miwa A, Okado H, Kiyama H: Akt/PKB prevents injury-induced motor neuron death and accelerates axonal regeneration. J Neurosci 20: 2875-2886, 2000 Kato H, Allen N, Emson PC, Kiyama H: GAP-43 N-terminal translocation signal targetss beta-galactosidase to developing axons in a pan-neuronal transgenic mouse line. Dev Brain Res 121: 109-112, 2000 Kiryu-Seo S, Sasaki M, Yokohama H, Nakagomi S, Hirayama T, Aoki S, Wada K, Kiyama H: Damage induced neuronal endopeptidase (DINE) is a unique metallopeptidase expressed in response to neuronal damage and activates superoxide scavengers. Proc Natl Acad Sci USA 97: 4345-4350, 2000 Takano R, Hisahara S, Namikawa K, Kiyama H, Okano H, Miura M: Nerve growth factor protects oligodendrocytes from TNF-α-induced injury through Akt-mediated signaling mechanisms. J Biol Chem 275: 16360-16365, 2000 Takeda M, Kato H, Takamiya A, Yoshida A, Kiyama H: Injury specific expression of activating transcription factor-3 (ATF-3) in nerve injured retinal ganglion cells, and its co-localized expression with phosphorylated c-Jun. Invest Ophth Vis Sci 41: 2412-2421, 2000 Tanabe K, Tachibana T, Yamashita T, Che YH, Yoneda Y, Ochi T, Tohyama M, Yoshikawa T, Kiyama H: The small GTP-binding protein TC10 promotes growth cone-like formation and nerve elongation in neuronal cells, and its expression is induced during nerve regeneration in rats. J Neurosci 20: 4138-4144, 2000 Nakagomi S, Kiryu-Seo S, Kiyama H: Endothelin converting enzymes and endothelin receptor B mRNAs are expressed in different neural cell species and these mRNAs are coordinately induced in neurons and astrocytes respectively following nerve injury. Neuroscience 101: 441-449, 2000 Gesase AP, Kiyama H: Morphological changes and expression of protein kinase CK2 beta subunit in the microglia after hypoglossal nerve transection. J Neurocytol 29: 61-66, 2000 Ishida-Yamamoto A, Kato H, Kiyama H, Armstrong DK, Munro CS, Eady RA, Nakamura S, Kinouchi M, Takahashi H, Iizuka H: Mutant loricrin is not crosslinked into the cornified cell envelope but is translocated into the nucleus in loricrin keratoderma. J Invest Dermatol 115: 1088-1094, 2000 Takeda M, Takamiya A, Yoshida A,Kiyama H: Extracellular signal-regulated kinase activation predominantly in Muller cells of retina with endotoxin-induced uveitis. Invest Ophth Vis Sci 43: 907-911, 2002 Takahashi H, Honma M, Ishida-Yamamoto A, Namikawa K, Miwa A, Okado H, Kiyama H, Iizuka H: In vitro and in vivo transfer of bcl-2 gene into keratinocytes suppresses UVB-induced apoptosis. Photochem Photobiol 74: 579-586, 2001 Takahashi H, Honnma M, Ishida-Yamamoto A, Namikawa K,Kiyama H, Iizuka H: Expression of human cystatin A by keratinocytes is positively regulated via the Ras/MEKK1/MKK7/JNK signal transduction pathway but negatively regulated via the Ras/Raf-1/MEK1/ERK pathway.J Biol Chem276: 36632-36638, 2001 Takamiya A, Takeda M, Yoshida A, Kiyama H: Expression of serine protease inhibitor 3 in ocular tissues in endotoxin induced uveitis in rat. Invest Ophth Vis Sci 42: 2427-2433, 2001 Iida N, Namikawa K, Kiyama H, Ueno H, Nakamura S, Hattori S: Requirement of Ras for the activation of mitogen-activated protein (MAP) kinase by calcium influx, cAMP and neurotrophin in hippocampal neurons. J Neurosci 21: 6459-6466, 2001 Takeda M, Mori F, Yoshida A, Takamiya A, Nakagomi S, Sato E, Kiyama H: Constitutive nitricoxide synthase is associated with retinal vascular permeability in early diabetic rats. Diabetologia44: 1043-1050, 2001 Matsuzaki H, Tamatani M, Yamaguchi A, Namikawa K, Kiyama H, Vitek MP, Mitsuda N, Tohyama M: Vascular endothelial growth factor rescues hippocampal neurons from glutamate-induced toxicity: signal transduction cascades. FASEB J 15: 1218-1220, 2001 Shirotani K, Tsubuki S, Iwata N, Takaki Y, Harigaya W, Maruyama K, Kiryu-Seo S, Kiyama H, Iwata H, Tomita T, Iwatsubo T, Saido TC: Neprilysin degrades both amyloid β peptides 1-40 and 1-42 most rapidly and efficiently among thiorphan- and phosphoramidon-sensitive endopeptidases J Biol Chem 276: 21895-21901, 2001 Abe K, Namikawa K, Honma M, Iwata T, Matsuoka I, Watabe K,Kiyama H: Inhibition of Ras-extracellular signal-regulated kinase (ERK) mediated signaling promotes ciliary neurotrophic factor (CNTF) expression in Schwann cells. J Neurochem 77: 700-703, 2001 Sasaki M, Seo-Kiryu S, Kato R, Kita S, Kiyama H: A disintegrin and metalloprotease with thrombospondin type1 motifs (ADAMTS-1) and IL-1 receptor type 1 mRNAs are simultaneously induced in nerve injured motor neurons. Mol Brain Res 89: 158-163, 2001 Mitsuda N, Ohkubo N, Tamatani M, Lee YD, Taniguchi M, Namikawa K, Kiyama H, Yamaguchi A, Sato N, Ogihara T, Vitek MP, Tohyama M: Activated CREB regulates neuronal expression of presenilin-1. J Biol Chem 276: 9688-9698, 2001 Yamaguchi A, Tamatani M, Matsuzaki H, Namikawa K, Kiyama H, Vitek MP, Mitsuda N, Tohyama M: Akt activation protects hippocampal neurons from apoptosis by inhibiting transcriptional activity of p53. J Biol Chem 276: 5256-5264, 2001 Kato R, Kiryu-Seo S, Kiyama H: Damage induced neuronal endopeptidase (DINE/ECEL) expression is regulated by LIF and deprivation of NGF in rat sensory ganglia after nerve injury. J Neurosci 22: 9410-9418, 2002 Aoki S, Su QN, Hang L, Nishikawa K, Ayukawa K, Hara Y, Namikawa K, Kiryu-Seo S, Kiyama H, Wada K: Identification of a novel axotomy-induced glycosylated protein, AIGP1, possibly involved in cellular apoptosis triggered by ER-Golgi stress. J Neurosci 22: 10751-10760, 2002 Honma M, Namikawa K, Mansur K, Iwata T, Mori N, Iizuka H, Kiyama H: Developmental alteration of nerve injury induced glial cell line-derived neurotrophic factor (GDNF) receptor expression is crucial for the determination of injured motoneuron fate. J Neurochem 82: 961-976, 2002 Iwata T, Namikawa K, Honma M, Mori N, Yachiku S, Kiyama H: Increased expression of mRNAs for microtubule disassembly molecules during nerve regeneration. Mol Brain Res 102: 105-109, 2002 Takamiya A, Takeda M, Yoshida A, Kiyama H: Inflammation induces serine protease inhibitor 3 (SPI-3) expression in the rat pineal gland. Neuroscience 113: 387-394, 2002 Osaka H, Wang YL, Takada K, Takizawa S, Setsuie R, Li H, Sato Y, Nishikawa K, Sun YJ, Sakurai M, Harada T, Hara Y, Kimura I, Chiba S, Namikawa K,Kiyama H, Noda M, Aoki S, Wada K: Ubiquitin carboxy-terminal hydrolase L1 binds to and stabilizes monoubiquitin in neuron. Hum Mol Genet 12: 1945-1958, 2003 KatoR, Kiryu-SeoS, SatoY, HisasueS, TsukamotoT, KiyamaH: Cavernous nerve injury elicits GAP-43 mRNA expression but not regeneration of injured pelvic ganglion neurons. Brain Res 986: 166-173, 2003 Suzuki Y, Nakagomi S, Namikawa K, Kiryu-Seo S, Inagaki N, Kaibuchi K, Aizawa H, Kikuchi K, Kiyama H: Collapsin response mediator protein-2 accelerates axon regeneration of nerve-injured motor neurons of rat. J Neurochem 86: 1042-1050, 2003 Ohba N, Maeda M, Nakagomi S, Muraoka M, Kiyama H: Biphasic expression of activating transcription factor-3 in neurons after cerebral infarction. Mol Brain Res 115: 147-156, 2003 Nakagomi S, Suzuki Y, Namikawa K, Kiryu-Seo S, Kiyama H: Expression of the activating transcription factor 3 prevents c-Jun N-terminal kinase-induced neuronal death by promoting heat shock protein 27 expression and Akt activation. J Neurosci 23: 5187-5196, 2003 小池　達郎　(北海道大学・大学院理学研究科) Mitsui C, Sakai K, Ninomiya T, Koike T: Involvement of TLCK-sensitive serine protease in colchicine-induced cell death of sympathetic neurons in culture. J Neurosci Res 66: 601-611, 2001 Saitoh T, Tanaka S, Koike T: Rapid unduction and Ca(2+) influx-mediated suppression of vitamin D3 up-regulated rotain 1 (VDUP1) mRNA in cerebellar granule neurons undergoing apoptosis. J Neurochem 78: 1267-1276, 2001 Tanaka S, Koike T: Activation of protein kinase C delays apoptosis of nerve growth factor-deprived rat sympathetic neurons through a Ca(2+)-influx dependent mechanism. Neurosci Lett 313: 9-12, 2001 Kaya N, Tanaka S, Koike T: ATP selectively suppresses the synthesis of the inflammatory protein microglial response factor (MRF)-1 through Ca(2+) influx via P2X(7) receptors in cultured microglia. Brain Res 952: 86-97, 2002 Tanaka S, Koike T: Selective inflammatory stimulations enhance release of microglial response factor (MRF)-1 from cultured microglia. Glia 40: 360-371, 2002 小坂　淳　(岡山大学・大学院医歯学総合研究科) Shibuta S, Kosaka J, Inoue T, Shimizu T, Tomi K, Mashimo T: The influence of the timing of administration of thiopentone sodium on nitric oxide-mediated neurotoxicity in vitro. J Neurol Sci 174: 9-15, 2000 Wakabayashi T, Kosaka J, Hommura S.: Up regulation of Hrk, a regulator of cell death, in retinal ganglion cells of axotomized rat retina. Neurosci Lett 318: 77-80, 2002 酒井　規雄　(広島大学・大学院医歯薬学総合研究科) Matsubayashi H, Amano T, Seki T, Sasa M, Sakai N: Postsynaptic α4 β2 and α7 type nicotinic acetylcholine receptors (nAChRs) contribute to the local and endogenous acetylcholine-mediated synaptic transmissions in nigral dopaminergic neurons. Brain Res 1005: 1-8, 2004 Uchino M, Sakai N, Kashiwagi K, Shirai Y, Shinohara Y, Hirose K, Iino M, Yamamura T, Saito N: Isoform-specific phosphorylation of mGluR5 by PKC blocks Ca2+ oscillation and oscillatory translocation of Ca2+-dependent PKC. J Biol Chem 279: 2254-2261, 2004 Seki T, Matsubayashi H, Amano T, Kitada K, Serikawa T, Sasa M, Sakai N: Adenoviral gene transfer of aspartoacylase ameliorates tonic convulsions of spontaneously epileptic rats. Neurochem Int 45: 171-178, 2004 Kajimoto T, Shirai Y, Sakai N, Yamamoto T, Matsuzaki H, Kikkawa U, Saito N: Ceramide-induced apoptosis by translocation, phosphorylation and activation of protein kinase Cδ at Golgi complex. J Biol Chem 279: 12668-12676, 2004 Yagi K, Shirai Y, Hirai M, Sakai N, Saito N: Phospholipase A2 products retain a neuron specific γ isoform of PKC on the plasma membrane through the C1 domain -A molecular mechanism for sustained enzyme activity. Neurochem Int 45: 39-47, 2004 Bhattacharjee AK, Ueyama T, Kondoh T, Hayashi S, Abouelfetouh A, Sakai N, Saito N, Kohmura E: In vivo transgene expression using an adenoviral tetracycline-regulated system with neuron-specific enolase promoter. Biochem Biophys Res Commun 317: 1144-1148, 2004 Amano T, Aihua Z, Matsubayasi H, Seki T, Serikawa T, Sasa M, Sakai N: Antiepileptic effects of single and repeated oral administrations of S-312-d, a novel calcium channel antagonist, on tonic convulsions in spontaneously epileptic rats (SER). J Pharmacol Sci 95: 355-362, 2004 Matsubayashi H, Inoue A, Amano T, Seki T, Nakata Y, Sasa M, Sakai N: Involvement of alpha7- and alpha4beta2 type postsynaptic nicotinic acetylcholine receptors in nicotine-induced excitation of dopaminergic neurons in the substantia nigra: A patch clamp and single-cell PCR study using acutely dissociated nigral neurons. Mol Brain Res 129: 1-7, 2004 Sakai N, Tsubokawa H, Matsuzaki M, Kajimoto T, Takahashi E, Ren Y, Ohmori S, Shirai Y, Matsubayashi H, Chen J, Duman RS, Kasai H, Saito N: Propagation of γPKC translocation along the dendrites of Purkinje cell in γPKC-GFP transgenic mice. Genes Cells 9: 945-957, 2004 Ueyama T, Lennartz MR, Noda Y, Kobayashi T, Shirai Y, Rikitake K, Yamasaki T, Hayashi S, Sakai N, Seguchi H, Sawada M, Sumimoto H, Saito N: Superoxide production at phagosomal cup/phagosome through beta I protein kinase C during Fc gamma R-mediated phagocytosis in microglia. J Immunol 173: 4582-4589, 2004 Mochizuki H, Amano T, Seki T, Matsubayashi H, Mitsuhata C, Morita K, Kitayama S, Dohi T, Mishima HK, Sakai N: Role of C-terminal region in the functional regulation of rat serotonin transporter (SERT). Neurochem Int 46: 93-105, 2005 Seki T, Matsubayashi H, Amano T, Shirai Y, Saito N, Sakai N: Phosphorylation of PKC activation loop plays an important role in receptor-mediated translocation of PKC. Genes Cells 10: 225-239, 2005 Fukunaga-Takenaka R, Shirai Y, Yagi K, Adachi N, Sakai N, Merino E, Merida I, Saito N: Importance of chroman ring and tyrosine phosphorylation in the subtype-specific translocation and activation of diacylglycerol kinase α by d-α-tocopherol. Genes Cells 10: 311-319, 2005 Abdel-Raheem IT, Hide I, Yanase Y, Shigemoto-Mogami Y, Sakai N, Shirai Y, Saito N, Hamada FM, El-Mahdy NA, Elsisy Ael-D, Sokar SS, Nakata Y: Protein kinase Cα mediates TNF release process in RBL-2H3 mast cells. Br J Pharmacol 145: 415-423, 2005 Kodama N, Murata Y, Asakawa A, Inui A, Hayashi M, Sakai N, Nanba H: Maitake D-Fraction enhances antitumor effects and reduces immunosuppression by mitomycin-C in tumor-bearing mice. Nutrition 21: 624-629, 2005 坂口　末廣　(長崎大学・大学院医歯学総合研究科) Li A, Sakaguchi S, Shigematsu K, Atarashi R, Roy BC, Nakaoke R, Arima K, Okimura N, Kopacek J, Katamine S: Physiological expression of the gene for PrP-like protein, PrPLP/Dpl, by brain endothelial cells and its ectopic expression in neurons of PrP-deficient mice ataxic to Purkinje cell degeneration. Am J Pathol 157: 1447-1452, 2000 Li A, Sakaguchi S, Atarashi R, Roy BC, Nakaoke R, Arima K, Okimura N, Kopacek J, Shigematsu K: Identification of a novel gene encoding a PrP-like protein expressed as chimeric transcripts fused to PrP exon 1/2 in ataxic mouse line with a disrupted PrP gene. Cell Mol Neurobiol 20: 553-567, 2000 Valenti P, Cozzio A, Nishida N, Wolfer DP, Sakaguchi S, Lipp HP: Similar target, different effects: late-onset ataxia and spatial learning in prion protein-deficient mouse lines. Neurogenetics 3: 173-184, 2001 Atarashi R, Sakaguchi S, Shigematsu K, Arima K, Okimura N, Yamaguchi N, Li A, Kopacek J, Katamine S: Abnormal activation of glial cells in the brains of prion protein-deficient mice ectopically expressing prion protein-like protein, PrPLP/Dpl. Mol Med 7: 803-809, 2001 Atarashi R, Nishida N, Shigematsu K, Goto S, Kondo T, Sakaguchi S, Katamine S: Deletion of N-terminal residues 23-88 from prion protein (PrP) abrogates the potential to rescue PrP-deficient mice from PrP-like protein/Doppel-induced neurodegeneration. J Biol Chem 278: 28944-28949, 2003 Yamaguchi N, Sakaguchi S, Shigematsu K, Okimura N, Katamine S: Doppel-induced Purkinje cell death is stoichiometrically abrogated by prion protein. Biochem Biophys Res Commun 319: 1247-1252, 2004 佐々木　秀直　(北海道大学・大学院医学研究科) Yabe I, Sasaki H, Takeichi N, Takei A, Hamada T, Fukushima K, Tashiro K: Positional vertigo and macroscopic downbeat positioning nystagmus in spinocerebellar ataxia type 6 (SCA6). J Neurol 250: 440-443, 2003 Yabe I, Sasaki H, Chen D, Wendy H. Raskind, Thomas D. Bird, Yamashita I, Tsuji S, Kikuchi S, Tashiro K： Spinocerebellar ataxia type 14 caused by a mutation in protein kinase Cγ. Arch Neurol 60: 1749-1751, 2003 佐野　輝　(愛媛大学・医学部) Suzuki A, Kondo T, Otani K, Mihara K, Yasui-Furukori N, Sano A, Koshiro K, Kaneko S:Association of the TaqI A polymorphism of the dopamine D (2) receptor gene with predisposition to neuroleptic malignant syndrome. Am J Psychiat 158: 1714-1716, 2001 Ueno S, Maruki Y, Nakamura M, Tomemori Y, Kamae K, Tanabe H, Yamashita Y, Matsuda S, Kaneko S, Sano A: The gene encoding a newly discovered protein, chorein, is mutated in chorea-acanthocytosis. Nat Genet28: 121-122, 2001 Sakai K, Nakamura M, Ueno S, Sano A, Sakai N, Shirai Y, Saito N: The silencer activity of the novel human serotonin transporter linked polymorphic regions. Neurosci Lett 327: 13-16, 2002 Sano A, Mikami M, Nakamura M, Ueno S, Tanabe H, Kaneko S: Positional candidate approach for the gene responsible for benign adult familial myoclonic epilepsy. Epilepsia 43 Suppl 9: 26-31, 2002 佐野　裕美　(京都大学・大学院医学研究科・先端領域融合医学研究機構) Nagai Y, Sano H, Yokoi M：Transgenic expression of Cre recombinase in mitral/tufted cells of the olfactory bulb. Genesis 43: 12-16, 2005 柴田　昌宏　(大阪大学・大学院医学系研究科) Ohsawa Y, Zhang G, Kametaka S, Shibata M, Koike M, Waguri S, Uchiyama Y: Purification, cDNA cloning, and secretory properties of FLRG protein from PC12 cells and the distribution of FLRG mRNA and protein in rat tissues. Arch Histol Cytol 66: 367-381, 2003 Zhang G, Ohsawa Y, Kametaka S, Shibata M, Waguri S, Uchiyama Y: Regulation of FLRG expression in rat primary astroglial cells and injured brain tissue by transforming growth factor-beta 1 (TGF-beta 1). J Neurosci Res 72: 33-45, 2003 Kametaka S, Shibata M, Moroe K, Kanamori S, Ohsawa Y, Waguri S, Sims PJ, Emoto K, Umeda M, Uchiyama Y: Identification of phospholipid scramblase 1 as a novel interacting molecule with beta-secretase (beta-site amyloid precursor protein (APP) cleaving enzyme (BACE)). J Biol Chem 278: 15239-15245, 2003 鈴木　俊顕　(東京都臨床医学総合研究所) Park KC, Choi EJ, Min SW, Chung SS, Kim H, Suzuki T, Tanaka K, Chung CH: Tissue specificity, functional characterization, and subcellular localization of rat ubiquitin-specific protease, UBP109, whose mRNA expression is developmentally regulated. Biochem J 349: 443-453, 2000 Shimura H, Hattori N, Kubo S, Mizuno Y, Asakawa S, Minoshima S, Shimizu N, Iwai K, Chiba T, Tanaka K, Suzuki T: Familial Parkinson’s disease gene product, parkin, is a ubiquitin-protein ligase. Nat Genet 25: 302-305, 2000 Osaka F, Saeki M, Aida N, Toh-e A, Katayama S, Kominami K, Toda T, Suzuki T, Chiba T, Tanaka K, Kato S: Covalent modifier NEDD8 is essential for SCF ubiquitin-ligase in fission yeast. EMBO J 19: 1-10, 2000 Kim KI, Baek SH, Jeon YJ, Nishimori S, Suzuki T, Uchida S, Shimbara N, Saitoh H, Tanaka K, Chung CH: A new SUMO-1-specific protease, SUSP1, that is highly expressed in reproductive organs. J Biol Chem 275: 14102-14106, 2000 Suzuki H, Chiba T, Suzuki T, Fujita T, Ikenoue T, Omata M, Furuichi K, Shikama H, Tanaka K: Homodimer of two F-box proteins βTrCP1 or βTrCP2 binds to IκBα for signal-dependent ubiquitination. J Biol Chem 275: 2877-2884, 2000 Takabe W, Kodama T, Hamakubo T, Tanaka K, Suzuki T, Aburatani H, Matsukawa N, Noguchi N: Anti-atherogenic antioxidants regulate the expression and function of proteasome α-type subunits in human endothelial cells. J Biol Chem 276: 40497-40501, 2001 Wang M, Suzuki T, Kitada T, Asakawa S, Minoshima S, Shimizu N, Tanaka K, Mizuno Y, Hattori N: Developmental changes in the expression of parkin and UbcR7, a parkin-interacting and ubiquitin-conjugating enzyme, in rat brain. J Neurochem 77: 1561-1568, 2001 Niwa J, Ishigaki S, Doyu M, Suzuki T, Tanaka K, Sobue G: A novel centrosomal RING-finger protein, Dorfin, mediates ubiquitin ligase activity. Biochem Biophys Res Commun 281: 706-713, 2001 Matsuda N, Suzuki T, Tanaka K, Nakano A: Rma1, a novel RING-finger protein conserved from Arabidopsis to human, is a membrane-bound ubiquitin ligase. J Cell Sci 114: 1949-1957, 2001 Kawakami T, Chiba T, Suzuki T, Iwai K, Yamanaka K, Minato N, Hidaka Y, Shimbara N, Suzuki H, Osaka. F, Omata M, Tanaka K: NEDD8 recruits E2-ubiquitin to SCF E3-ligase. EMBO J 20: 4003-401, 2001 Yoshida Y, Chiba T, Tokunaga F, Kawasaki H, Iwai K, Suzuki T, Ito Y, Matsuoka K, Yoshida M, Tanaka K, Tai T: E3 ubiquitin ligase that recognizes sugar chains. Nature 418: 438-442, 2002 Kadoya T, Yamamoto H, Suzuki T, Yukita A, Fukui A, Michiue T, Asahara T, Tanaka K, Asashima M, Kikuchi A: Desumoylation activity of Axam, a novel Axin-binding protein, is involved in downregulation of beta-catenin. Mol Cell Biol 22: 3803-3819, 2002 Ogawara Y, Kishishita S, Obata T, Isazawa Y, Suzuki T, Tanaka K, Masuyama N, Gotoh Y: Akt enhances Mdm2-mediated ubiquitination and degradation of p53. J Biol Chem 277: 21843-21850, 2002 Araki K, Kawamura M, Suzuki T, Matsuda N, Kanbe D, Ishii K, Ichikawa T, Kumanishi T, Chiba T, Tanaka K, Nawa H: A palmitoylated RING finger ubiquitin ligase and its homologue, boh enriched in the brain membranes. J Neurochem 86: 749-762, 2003 高橋　良輔　(理化学研究所・脳科学総合研究センター) Kawata A, Kato S, Shimizu T, Hayashi H, Hirai S, Misawa H, Takahashi R: Aberrant splicing of human Cu/Zn superoxide dismutase (SOD1) RNA transcripts. NeuroReport 11: 2649-2653, 2000 Imai Y, Soda M, Takahashi R: Parkin suppresses unfolded protein stress-induced cell death through its E3 ubiquitin-protein ligase activity. J Biol Chem 275: 35661-35664, 2000 Suzuki Y, Nakabayashi Y, Nakata K, Reed JC, Takahashi R: X-linked inhibitor of apoptosis protein (XIAP) inhibits caspase-3 and -7 in distinct modes. J Biol Chem 276: 27058-27063, 2001 Suzuki Y, Nakabayashi Y, Takahashi R: Ubiquitin-protein ligase activity of X-linked inhibitor of apoptosis protein promotes proteasomal degradation of caspase-3 and enhances its anti-apoptotic effect in Fas-induced cell death. Proc Natl Acad Sci USA 98: 8662-8667, 2001 Imai Y, Soda M, Inoue H, Hattori N, Mizuno Y, Takahashi R: An unfolded putative membrane transmembrane polypeptide, which can lead to endoplasmic reticulum stress, is a substrate of Parkin. Cell 105: 891-902, 2001 Suzuki Y, Imai Y, Nakayama H, Takahashi K, Takio K, Takahashi R: Aserine protease, HtrA2, is released from the mitochondria and interacts with XIAP, inducing cell death. Mol Cell 8: 613-621, 2001 Ishida K, Takeuchi H, Takahashi R, Yoshimura K, Yamada M, Mizusawa H: A possible novel isoform of peripheral myelin P0 protein: a target antigen recognized by an autoantibody in a patient with malignant lymphoma and peripheral neuropathy. J Neurol Sci 188: 43-49, 2001 Araya R, Takahashi R, Nomura Y: Yeast two-hybrid screening using constitutive-active caspase-7 as bait in the identification of PA28γ as an effector caspase substrate. Cell Death Differ 9: 322-328, 2002 Imai Y, Soda M, Hatakeyama S, Akagi T, Hashikawa T, Nakayama KI, Takahashi R: CHIP is associated with Parkin, a gene responsible for familial Parkinson‘s disease, and enhances its ubiquitin ligase activity. Mol Cell 10: 55-67, 2002 Kobayashi S, Yamashita K, Takeoka T, Ohtsuki T, Suzuki Y, Takahashi R, Yamamoto K, Kaufmann SH, Uchiyama T, Sasada M, Takahashi A: Calpain-mediated X-linked inhibitor of apoptosis degradation in neutrophil apoptosis and its impairment in chronic neutrophilic leukemia. J Biol Chem 277: 33968-33977, 2002 Urushitani U, Kurisu J, Tsukita K, Takahashi R: Proteasomal inhibition by misfolded mutant superoxide dismutase 1 induces selective motor neuron death in familial amyotrophic lateral sclerosis. J Neurochem 83: 1030-1042, 2002 Imai Y, Soda M, Murakami T, Shoji M, Abe K, Takahashi R: A product of the human gene adjacent to parkin is a component of Lewy bodies and suppresses Pael receptor-induced cell death. J Biol Chem 278: 51901-51910, 2003 Inoue H, Tsukita K, Iwasato T, Suzuki Y, Tomioka M, Tateno M, Nagao M, Kawata A, Saido TC, Miura M, Misawa H, Itohara S, Takahashi R: The crucial role of caspase-9 in the disease progression of transgenic ALS mouse model. EMBO J 22: 6665-6674, 2003 Yokota T, Sugawara K, Takahashi R, Ariga H, Mizusawa H: Down regulation of DJ-1 enhances the cell death by oxidative stress, ER-stress and proteasome inhibition. Biochem Biophys Res Commun 312: 1342-1348, 2003 Yang Y, Nishimura I, Imai Y, Takahashi R, Lu B: Parkin suppresses dopaminergic neuron-selective neurotoxicity induced by Pael-R in Drosophila. Neuron 37: 911-924, 2003 Misawa H, Nakata K, Toda K, Matsuura J, Oda Y, Inoue H, Taneno M, Takahashi R: VAChT-Cre.Fast and VAChT-Cre.Slow: postnatal expression of Cre recombinase in somatomotor neurons with different onset. Genesis 37: 44-50, 2003 Suzuki Y, Takahashi-Niki K, Akagi T, Hashikawa T, Takahashi R: The mitochondrial protease Omi-HtrA2 enhances caspase activation through multiple pathways. Cell Death Differ 11: 208-216, 2004 Yokota T, Miyagishi M, Hino T, Matsumura R, Andria T, Urushitani M, Rao RV, Takahashi R, Bredesen DE, Taira K, Mizusawa H: siRNA-based inhibition specific for mutant SOD1 with single nucleotide alternation in familial ALS, compared with ribozyme and DNA enzyme. Biochem Biophys Res Commun 314: 283-291, 2004 Murakami T, Shoji M, Imai Y, Inoue H, Kawarabayashi T, Matsubara E, Harigaya Y, Sasaki A, Takahashi R, Abe K: Pael-R is accumulated in Lewy bodies of Parkinson’s disease. Ann Neurol 55: 439-442, 2004 Urushitani M, Kurisu J, Tateno M, Hatakeyama S, Nakayama KI, Kato S, Takahashi R: CHIP promotes proteasomal degradation of familial ALS-linked mutant SOD1 by ubiquitinating Hsp/Hsc70. J Neurochem 90: 231-244, 2004 Hosokawa Y, Suzuki H, Suzuki Y, Takahashi R, Seto M: Anti-apoptotic function of API2-MALT1 fusion protein involved in t(11;14)(q21;q21) MALT lymphoma. Cancer Res 64: 3452-3457, 2004 Vyas S, Juin P, Hancock D, Suzuki Y, Takahashi R, Triller A, Evan G: Differentiation dependent sensitivity to apoptogenic factors in PC12 cells. J Biol Chem 279: 30983-30993, 2004 Tateno M, Sadakata H, Tanaka M, Itohara S, Shin RM, Miura M, Masuda M, Aosaki T, Urushitani M, Misawa H, Takahashi R: Calcium-permeable AMPA receptors promote misfolding of mutant SOD1 protein and development of amyotrophic lateral sclerosis in a transgenic mouse model. Hum Mol Genet 13: 2183-2196, 2004 Yamamoto A, Friedlein A, Imai Y, Takahashi R, Kahle PJ, Haass C: Parkin phosphorylation and modulation of its E3 ubiquitin ligase activity. J Biol Chem 280: 3390-3399, 2005 Hatakeyama S, Matsumoto M, Kamura T, Murayama M, Chui DH, Planel E, Takahashi R, Nakayama KI, Takashima A: U-box protein carboxyl terminus of Hsc70-interacting protein (CHIP) mediates poly-ubiquitylation preferentially on four-repeat Tau and is involved in neurodegeneration of tauopathy. J Neurochem 91: 299-307, 2004 Kim YJ, Nakatomi R, Akagi T, Hashikawa T, Takahashi R: Unsaturated fatty acids induce cytotoxic aggregate formation of amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutants. J Biol Chem 280: 21515-21521, 2005 田邊　勉　（東京医科歯科大学・大学院医歯学総合研究科） Kurihara T, Nonaka T, Tanabe T: Acetic acid-conditioning stimulus induces long-lasting antinociception of somatic inflammatory pain. Pharmacol Biochem Behav74: 841-849, 2003 Takei T, Saegusa H, Zong S, Murakoshi T, Makita K, Tanabe T: Anesthetic sensitivities to propofol and halothane in mice lacking the R-type (Cav2.3) Ca2+ channel. Anesth Analg97: 96-103, 2003 Takei T, Saegusa H, Zong S, Murakoshi T, Makita K, Tanabe T: Increased sensitivity to halothane but decreased sensitivity to propofol in mice lacking the N-type Ca2+ channel. Neurosci Lett350: 41-45, 2003 Yokoyama K, Kurihara T, Makita K, Tanabe T: Plastic change of N-type Ca channel expression after preconditioning is responsible for prostaglandin E2-induced long-lasting allodynia. Anesthesiology 99: 1364-1370, 2003 Takahashi E, Fukuda K, Miyoshi S, Murta M, Kato T, Ita M, Tanabe T, Ogawa S: Leukemia inhibitory factor activates cardiac L-type Ca2+ channels via phosphorylation of serine 1829 in the rabbit CaV1.2 subunit. Circ Res 94: 1242-1248, 2004 Yokoyama K, Kurihara T, Saegusa H, Zong S, Makita K, Tanabe T: Blocking the R-type (CaV2.3) Ca2+ channel enhanced morphine analgesia and reduced morphine tolerance. Eur J Neurosci 20: 3516-3519, 2004 程　久美子　（東京大学・大学院理学系研究科） Hamada T, Ui-Tei K, Imaki J, Miyata Y: Molecular cloning of SCDGF-B, a novel growth factor homologous to SCDGF/PDGF-C/fallotein. Biochem Biophys Res Commun 280: 733-737, 2001 Piao Z-f, Ui-Tei K, Nagano M, Miyata Y: Participation of intracellular Ca2+/calmodulin and protein kinase(s) in the pathway of apoptosis induced by a Drosophila cell death gene. Mol Cell Biol Res Commun 4: 307-312, 2001 Hamada T, Ui-Tei K, Imaki J, Takahashi F, Onodera H, Mishima T, Miyata Y: The expression of SCDGF/PDGF-C/fallotein and SCDGF-B/PDGF-D in the rat central nervous system. Mech Dev 112: 161-164, 2002 Seike M, Gemma A, Hosoya Ｙ, Hosomi Y, Okano T, Kurimoto F, Uematsu K, Tekenaka K, Yoshimura A, Shibuya M, Ui-Tei K, Kudoh S: The pomoter region of the human BUBR1 gene and its expression analysis in lung cancer. Lung Cancer 38: 229-234, 2002 Uwo M.F, Ui-Tei K, Park P, Takeda M: Replacement of midgut epithelium in the greater wax moth, Galleria mellonela, during larval-pupal moult. Cell Tissue Res 308: 319-331, 2002 Doi N, Zenno S, Ueda R, Ohki-Hamazaki H, Ui-Tei K, Saigo K: Short-interfering-RNA-mediated gene silencing in mammalian cells requires Dicer and eIF2C translation initiation factors. Curr Biol 13: 41-46, 2003 Iwabushi M, Ui-Tei K, Yamada K, Matsuda Y, Sakai Y, Tanaka K, Ohki-Hamazaki H: Molecular cloning and characterization of avian bombesin-like peptide receptors: New tools for investigating molecular bais for ligand selectivity. Br J Pharmacol 139: 555-566, 2003 Kato M, Ui-Tei K, Watanabe M, Sakuma Y: Characterization of voltage-gated calcium currents in gonadotropin-releasing hormone neurons tagged with green fluorescent protein in rats. Endocrinology 144: 5118-5125, 2003 Mishima T, Hamada T, Ui-Tei K, Takahashi F, Miyata Y, Imaki J, Suzuki H, Yamashita K: Expression of DDAH1 in chick and rat embryos. Dev Brain Res 148: 223-232, 2004 Ui-Tei K, Ueda R, Zenno S, Takahashi F, Doi N, Naito Y, Yamamoto M, Hashimoto N, Takahashi K, Hamada T, Tokunaga T, Saigo K: RNA interference induced by transient or stable expression of hairpin structures of double stranded RNA in Drosophila and mammalian cells. Mol Biol (Mosk)38: 276-287, 2004 Ui-Tei K, Naito Y, Takahashi F, Haraguchi T, Ohki-Hamazaki H, Juni A, Ueda R, Saigo K: Guidelines for the selection of highly effective siRNA sequences for mammalian and chick RNA interference. Nucleic Acids Res 32: 936-948, 2004 Takagi Y, Momizu M, Ui-Tei K, Tokushige N, Hirohashi S: Active sites in the carboxyl-terminal region of the laminn a chain in Drosophila Neuronal cell spreading. Arch Insect Biochem Physiol 56: 162-169, 2004 Naito Y, Yamada T, Ui-Tei K, Morishita S, Saigo K: siDirect: highly effective, target-specific siRNA design software for mammalian RNA interference. Nucleic Acids Res 32: W124-W129, 2004. Niimura M, Isoo N, Takasugi N, Tsuruoka M, Ui-Tei K, Saigo K, Morohashi Y, Tomita T, Iwatsubo T: Aph-1 contributes to the stabilization and trafficking of the γ-secretase complex through mechanisms involving inter- and intramolecular interactions. J Biol Chem 280: 12967-12975, 2005 Takahashi M, Takahashi F, Ui-Tei K, Kojima T, Saigo K: Requirements of genetic interactions between Src42A, armadillo and shotgun, a gene encoding E-cadherin, for normal development in Drosophila. Development 132: 2547-2559, 2005 Naito Y, Yamada T, Matsumiya T, Ui-Tei K, Saigo K, Morishita S: dsCheck: highly sensitive off-target search software for dsRNA-mediated RNA interference. Nucleic Acids Res 33: W589-W591, 2005 遠山　正彌　（大阪大学・大学院医学系研究科） Yoneda T, Imaizumi K, Maeda M, Yui D, Manabe T, Katayama T, Sato N, Gomi F, Morihara T, Mori Y, Miyoshi K, Hitomi J, Ugawa S, Yamada S, Okabe M, Tohyama M: Regulatory mechanisms of TRAF2-mediated signal transduction by Bcl10, a MALT lymphoma-associated protein. J Biol Chem 275: 11114-11120, 2000 Taniguchi M, Yamashita T, Kumura E, Tamatani M, Kobayashi A, Yokawa T, Maruno M, Kato A, Ohnishi T, Kohmura E, Tohyama M, Yoshimine T: Induction of aquaporin-4 water channel mRNA after focal cerebral ischemia in rat. Mol Brain Res 78: 131-137, 2000 Tanabe K, Tachibana T, Yamashita T, Che Y. H., Yoneda Y, Ochi T, Tohyama M, Yoshikawa H, Kiyama H: The small GTP-binding protein TC10 promotes nerve elongation in neuronal cells, and its expression is induced during nerve regeneration in rats. J Neurosci 20: 4138-4144, 2000 Tamatani M, Mitsuda N, Matsuzaki H, Okado H, Miyake S, Vitek M. P., Yamaguchi A, Tohyama M: A pathway of neuronal apoptosis induced by hypoxia/reoxygenation: roles of nuclear factor-kappaB and Bcl-2. J Neurochem 75: 683-693, 2000 Takakuwa T, Endo S, Shirakura Y, Yokoyama M, Tamatani M, Tohyama M, Aozasa K, Inada K: Interleukin-10 gene transfer improves the survival rate of mice inoculated with Escherichia coli. Crit Care Med 28: 2685-2689, 2000 Morihara T, Katayama T, Sato N, Yoneda T, Manabe T, Hitomi J, Abe H, Imaizumi K, Tohyama M: Absence of endoproteolysis but no effects on amyloid beta production by alternative splicing forms of presenilin-1, which lack exon 8 and replace D257A. Mol Brain Res 85: 85-90, 2000 Mori Y, Imaizumi K, Katayama T, Yoneda T, Tohyama M: Two cis-acting elements in the 3' untranslated region of alpha-CaMKII regulate its dendritic targeting. Nat Neurosci 3: 1079-84, 2000 Miyoshi K, Katayama T, Imaizumi K, Taniguchi M, Mori Y, Hitomi J, Yui D, Manabe T, Gomi F, Yoneda T, Tohyama M: Characterization of mouse Ire1alpha: cloning, mRNA localization in the brain and functional analysis in a neural cell line. Mol Brain Res 85: 68-76, 2000 Kudo T, Imaizumi K, Tanimukai H, Katayama T, Sato N, Nakamura Y, Tanaka T, Kashiwagi Y, Jinno Y, Tohyama M, Takeda M: Are cerebrovascular factors involved in Alzheimer’s disease? Neurobiol Aging 21: 215-224, 2000 Horimoto M, Sasaki Y, Ugawa S, Wada S, Toyama T, Iyoda K, Yakushijin T, Minami Y, Ito T, Hijioka T, Eguchi A, Nakanishi M, Shimada S, Tohyama M, Hayashi N, Hori M: A novel strategy for cancer therapy by mutated mammalian degenerin gene transfer. Cancer Gene Ther 7: 1341-1347, 2000 Gomi F, Imaizumi K, Yoneda T, Taniguchi M, Mori Y, Miyoshi K, Hitomi J, Fujikado T, Tano Y, Tohyama M: Molecular cloning of a novel membrane glycoprotein, pal, specifically expressed in photoreceptor cells of the retina and containing leucine- rich repeat. J Neurosci 20: 3206-3213, 2000 Che YH, Tamatani M, Yamashita T, Gomi F, Ogawa S, Tohyama M: Changes in mRNA of protein inhibitor of neuronal nitric oxide synthase following facial nerve transection. J Chem Neuroanat 17: 199-206, 2000 Che YH, Tamatani M, Tohyama M: Changes in mRNA for post-synaptic density-95 (PSD-95) and carboxy- terminal PDZ ligand of neuronal nitric oxide synthase following facial nerve transection. Mol Brain Res 76: 325-335, 2000 Bando Y, Ogawa S, Yamauchi A, Kuwabara K, Ozawa K, Hori O, Yanagi H, Tamatani M, Tohyama M: 150-kDa oxygen-regulated protein (ORP150) functions as a novel molecular chaperone in MDCK cells. Am J Physiol Cell Physiol 278: C1172-C1182, 2000 Yui D, Yoneda T, Oono K, Katayama T, Imaizumi K, Tohyama M: Interchangeable binding of Bcl10 to TRAF2 and cIAPs regulates apoptosis signaling. Oncogene 20: 4317-4323, 2001 Yoneda T, Imaizumi K, Oono K, Yui D, Gomi F, Katayama T, Tohyama M: Activation of caspase-12, an endoplasmic reticulum (ER) resident caspase, through tumor necrosis factor receptor-associated factor 2- dependent mechanism in response to the ER stress. J Biol Chem 276: 13935-13940, 2001 Yara S, Kawakami K, Kudeken N, Tohyama M, Teruya K, Chinen T, Awaya A, Saito A: FTS reduces bleomycin-induced cytokine and chemokine production and inhibits pulmonary fibrosis in mice. Clin Exp Immunol 124: 77-85, 2001 Yamaguchi A, Tamatani M, Matsuzaki H, Namikawa K, Kiyama H, Vitek M. P., Mitsuda N, Tohyama M: Akt activation protects hippocampal neurons from apoptosis by inhibiting transcriptional activity of p53. J Biol Chem 276: 5256-5264, 2001 Utsumi M, Ohno K, Onchi H, Sato K, Tohyama M: Differential expression patterns of three glutamate transporters (GLAST, GLT1 and EAAC1) in the rat main olfactory bulb. Mol Brain Res 92: 1-11, 2001 Tamatani M, Matsuyama T, Yamaguchi A, Mitsuda N, Tsukamoto Y, Taniguchi M, Che YH, Ozawa K, Hori O, Nishimura H, Yamashita A, Okabe M, Yanagi H, Stern DM, Ogawa S, Tohyama M: ORP150 protects against hypoxia/ischemia-induced neuronal death. Nat Med 7: 317-323, 2001 Sato N, Imaizumi K, Manabe T, Taniguchi M, Hitomi J, Katayama T, Yoneda T, Morihara T, Yasuda Y, Takagi T, Kudo T, Tsuda T, Itoyama Y, Makifuchi T, Fraser PE, St George-Hyslop P, Tohyama M: Increased production of beta-amyloid and vulnerability to endoplasmic reticulum stress by an aberrant spliced form of presenilin 2. J Biol Chem 276: 2108-2114, 2001 Sakata K, Yamashita T, Maeda M, Moriyama Y, Shimada S, Tohyama M: Cloning of a lymphatic peptide/histidine transporter. Biochem J 356: 53-60, 2001 Ohkubo N, Mitsuda N, Tamatani M, Yamaguchi A, Lee Y. D., Ogihara T, Vitek M. P., Tohyama M: Apolipoprotein E4 stimulates cAMP response element-binding protein transcriptional activity through the extracellular signal-regulated kinase pathway. J Biol Chem 276: 3046-3053, 2001 Mitsuda N, Ohkubo N, Tamatani M, Lee YD, Taniguchi M, Namikawa K, Kiyama H, Yamaguchi A, Sato N, Sakata K, Ogihara T, Vitek MP, Tohyama M: Activated cAMP-response element-binding protein regulates neuronal expression of presenilin-1. J Biol Chem 276: 9688-9698, 2001 Matsuzaki H, Tamatani M, Yamaguchi A, Namikawa K, Kiyama H, Vitek MP, Mitsuda N, Tohyama M: Vascular endothelial growth factor rescues hippocampal neurons from glutamate-induced toxicity: signal transduction cascades. FASEB J 15: 1218-1220, 2001 Katayama T, Imaizumi K, Honda A, Yoneda T, Kudo T, Takeda M, Mori K, Rozmahel R, Fraser P, St George-Hyslop, P, Tohyama M: Disturbed activation of endoplasmic reticulum stress transducers by familial Alzheimer's disease-linked presenilin 1 mutations. J Biol Chem 276: 43446-43454, 2001 Inagaki S, Ohoka Y, Sugimoto H, Fujioka S, Amazaki M, Kurinami H, Miyazaki N, Tohyama M, Furuyama T: Sema4c, a transmembrane semaphorin, interacts with a post-synaptic density protein, PSD-95. J Biol Chem 276: 9174-9181, 2001 Imaizumi K, Katayama T, Tohyama M: Presenilin and the UPR. Nat Cell Biol 3: E104, 2001 Imaizumi K, Miyoshi K, Katayama T, Yoneda T, Taniguchi M, Kudo T, Tohyama M: The unfolded protein response and Alzheimer’s disease. Biochim Biophys Acta 1536: 85-96, 2001 中井　彰　（山口大学・大学院医学研究科） Nakai A, Suzuki M, Tanabe M: Arrest of spermatogenesis in mice expressing an active heat shock transcription factor 1. EMBO J 19: 1545-1554, 2000 Tanikawa J, Ichikawa-Iwata E, Kanei-Ishii C, Nakai A, Matsuzawa S, Reed JC, Ishii S: p53 suppresses the c-myb-induced activation of heat shock transcription factor 3. J Biol Chem 275: 15578-15585, 2000 Nakai A, Ishikawa T: A nuclear localization signal is essential for stress-induced dimer-to-trimer transition of heat shock transcription factor 3. J Biol Chem 275: 34665-34671, 2000 Nakai A, Ishikawa T: Cell cycle transition under stress conditions controlled by vertebrate heat shock factors. EMBO J 20: 2885-2895, 2001 Nishizawa J, Nakai A, Komeda M, Ban T, Nagata K: Increased preload directly induces the activation of heat shock transcription factor 1 in the left ventricular overloaded heart. Cardiovasc Res 55: 341-348, 2002 Shallom JM, Di Carlo AL, Ko D, Penafiel LM, Nakai A, Litovitz TA: Microwave exposure induces Hsp70 and confers protection against hypoxia in chick embryos. J Cell Biochem 86: 490-496, 2002 永井　義隆　（大阪大学・大学院医学系研究科） Tachikawa M, Nagai Y, Nakamura K, Kobayashi K, Fujiwara T, Han HJ, Nakabayashi Y, Ichikawa Y, Goto J, Kanazawa I, Nakamura Y, Toda T: Identification of CAG repeat-containing genes expressed in human brain as candidate genes for autosomal dominant spinocerebellar ataxias and other neurodegenerative diseases. J Hum Genet 47: 275-278, 2002 Azuma T, Uemichi T, Funauchi M, Nagai Y, Matsubara T: Ambulatory blood pressure monitoring in patients with spinocerebellar degeneration. Acta Neurol Scand 106: 213-217, 2002 Nagai Y, Fujikake N, Ohno K, Higashiyama H, Popiel HA, Rahadian J, Yamaguchi M, Strittmatter WJ, Burke JR, Toda T: Prevention of polyglutamine oligomerization and neurodegeneration by the peptide inhibitor QBP1 in Drosophila. Hum Mol Genet 12: 1253-1259, 2003 Toda T, Kobayashi K, Takeda S, Sasaki J, Kurahashi H, Kano H, Wang F, Tachikawa M, Nagai Y, Sunada Y, Terashima T, Endo T, Matsumura K: Fukuyama-type congenital muscular dystrophy (FCMD) and α-dystroglycanopathy. Congenit Anom 43: 97-104, 2003 Toda T, Kobayashi K, Takeda S, Sasaki J, Kurahashi H, Kano H, Tachikawa M, Wang F, Nagai Y, Taniguchi K, Taniguchi M, Sunada Y, Terashima T, Endo T, Matsumura K: Fukuyama-type congenital muscular dystrophy and abnormal glycosylation of α-dystroglycan. Basic Appl Myol 13: 287-292, 2003 Popiel HA, Nagai Y, Onodera O, Inui T, Fujikake N, Urade Y, Strittmatter WJ, Burke JR, Ichikawa A, Toda T: Disruption of the toxic conformation of the expanded polyglutamine stretch leads to suppression of aggregate formation and cytotoxicity. Biochem Biophys Res Commun 317: 1200-1206, 2004 Kariya S, Hirano M, Nagai Y, Furiya Y, Fujikake N, Toda T, Ueno S: Humanin attenuates apoptosis induced by DRPLA proteins with expanded polyglutamine stretches. J Mol Neurosci 25: 165-170, 2005 Chiyonobu T, Sasaki J, Nagai Y, Takeda S, Funakoshi H, Nakamura T, Sugimoto T, Toda T: Effects of fukutin deficiency in the developing mouse brain. Neuromuscul Disord 15: 416-426, 2005 Fujikake N, Nagai Y, Popiel HA, Kano H, Yamaguchi M, Toda T: Alternative splicing regulates the transcriptional activity of Drosophila heat shock transcription factor in response to heat/cold stress. FEBS Lett 579: 3842-3848, 2005 長野　精一　（大阪大学・大学院医学系研究科） Sugai F, Yamamoto Y, Miyaguchi K, Zhou Z, Sumi H, Hamasaki T, Goto M, Sakoda S: Benefit of valproic acid in suppressing disease progression of ALS model mice. Eur J Neurosci 20: 3179-3183, 2004 Nagano S, Huang X, Moir RD, Payton SM, Tanzi RE, Bush AI: Peroxidase activity of cyclooxygenase-2 (COX-2) cross-links beta-amyloid (Abeta) and generates Abeta-COX-2 hetero-oligomers that are increased in Alzheimer’s disease. J Biol Chem 279: 14673-14678, 2004 Sato T, Yamamoto Y, Nakanishi T, Fukada K, Sugai F, Zhou Z, Okuno T, Nagano S, Hirata S, Shimizu A, Sakoda S: Identification of two novel mutations in the Cu/Zn superoxide dismutase gene with familial amyotrophic lateral sclerosis: mass spectrometric and genomic analyses. J Neurol Sci 218: 79-83, 2004 Puglielli L, Friedlich AL, Setchell KDR, Nagano S, Opazo C, Cherny RA, Barnham KJ, Wade JD, Melov S, Kovacs DM, Bush AI: Cholesterol oxidase mimetic activity of Alzheimer’s disease beta-amyloid. J Clin Invest 115: 2556-2563, 2005 Nishioka N, Nagano S, Nakayama R, Kiyonari H, Ijiri T, Taniguchi K, Shawlot W, Hayashizaki Y, Westphal H, Behringer RR, Matsuda Y, Sakoda S, Kondoh H, Sasaki H: Ssdp1 regulates head morphogenesis of mouse embryos by activating the Lim1-Ldb1 complex. Development 132: 2535-2546, 2005 中村　浩一郎　（東京大学・医学部附属病院・神経内科） Nakamura K, Jeong, S-Y, Uchihara T, Anno M, Nagashima K, Nagashima T, Ikeda S, Tsuji S, Kanazawa I: SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein. Hum Mol Genet 10: 1441-1448, 2001 Hazeki N, Tsukamoto T, Yazawa I, Koyama M, Hattori S, Someki I, Iwatsubo T, Nakamura K, Goto J, Kanazawa I: Ultrastructure of nuclear aggregates formed by expressing an expanded polyglutamine. Biochem Biophys Res Commun 294: 429-440, 2002 Tachikawa M, Nagai Y, Nakamura K, Kobayashi K, Fujiwara T, Han HJ, Nakabayashi Y, Ichikawa Y, Goto J, Kanazawa I, Nakamura Y, Toda T: Identification of CAG repeat-containing genes expressed in human brain as candidate genes for autosomal dominant spinocerebellar ataxias and other neurodegenerative diseases. J Hum Genet 47: 275-278, 2002 中村　重信　（広島大学・医学部） Maruyama H, Izumi Y, Oda M, Torii T, Morino H, Toji H, Sasaki K, Terasawa H, Nakamura S, Kawakami H: Lack of an association of between cystatin C gene polymorphisms in Japanese patients with Alzheimer’s disease. Neurology 57: 337-339, 2001 Urakami K, Wada K, Arai H, Sasaki H, Kanai M, Shoji M, Ishizu H, Kashihara K, Yamamoto M, Tsuchiya-Ikemoto K, Morimatsu M, Takashima M, Nakagawa M, Kurokawa K, Maruyama H, Kaseda Y, Nakamura S, Hasegawa K, Oono H, Hikasa C, Ikeda K, Yamagata K, Wakutani Y, Takeshima T, Nakashima K: Diagnostic significance of tau protein in cerebrospinal fluid from patients with corticobasal degeneration or progressive supranuclear palsy. J Neurol Sci 183: 95-98, 2001 Nakamura T, Yamashita H, Takahashi T, Nakamura S: Activated fyn phosphorylates α-synuclein at tyrosine residue 125. Biochem Biophys Res Commun 280: 1085-1092, 2001 Izumi Y, Morino H, Oda M, Maruyama H, Udaka F, Kameyama M, Nakamura S, Kawakami H: Genetic studies in Parkinson’s disease with an α-synuclein/NACP gene polymorphism in Japan. Neurosci Lett 300: 125-127, 2001 Yukawa M, Naka H, Murata Y, Katayama S, Kohriyama T, Mimori Y, Nakamura S: Folic acid-responsive neurological diseases in Japan. J Nutr Sci Vitaminol 47: 181-187, 2001 Oka M, Katayama S, Imon Y, Ohshita T, Mimori Y, Nakamura S: Abnormal signals on proton density-weighted MRI of the superior cerebellar peduncle in progressive supranuclear palsy. Acta Neurol Scand 104: 1-5, 2001 Inoue K, Hashimoto I, Nakamura S: High-frequency oscillations in human posterior tibial somatosensory evoked potentials are enhanced in patients with parkinson’s disease and multiple system atrophy. Neurosci Lett 297: 89-92, 2001 Nishimura M, Kawakami H, Maruyama H, Izumi Y, Kuno S, Kaji R, Nakamura S: Influence of interleukin-1β gene polymorphism on age-at-onset of spinocerebellar ataxia 6 (SCA6). Neurosci Lett 307: 128-130, 2001 Kitae S, Murata Y, Tachiki N, Okazaki M, Harada T, Nakamura S: Assessment of cardiovascular autonomic dysfunction in multiple system atrophy. Clin Auton Res 11: 39-44, 2001 Kato M, Aoki M, Ohta M, Nagai M, Ishizaki F, Nakamura S, Itoyama Y: Maked reducution of the Cu/Zn superoxide dismutase polypeptide in a case of familial amyotrophic lateral sclerosis with the homozygous mutation. Neurosci Lett 312: 165-168, 2001 Harada T, Ishizaki F, Murata Y, Mitsuoka T, Kumagai R, Nitta K, Nakamura S: Clinical characteristics of neuroleptic malignant syndrome in Parkinson’s disease and other neurological disorders. Int Med J 8: 249-257, 2001 Harada T, Ishizaki F, Tachiki N, Nitta K, Horie N, Matsumoto T, Inoue K, Nakamura S: Evaluation of cardiovascular circadian rhythm in patients with Parkinson’s disease. Int Med J 8: 263-268, 2001 Harada T, Ishizaki F, Murata Y, Nitta K, Tachiki N, Okazaki M, Nakamura S: Circadian rhythm of blood pressure and pulse rate in diabetes mellitus. Int Med J 8: 281-288, 2001 Mao JJ, Katayama S, Watanabe C, Harada Y, Noda K, Yamamura Y, Nakamura S: The relationship of αB-crystallin and neurofibrillary tangles in Alzheimer’s disease. Neuropathol Appl Neurobiol 27: 180-188, 2001 Nagano Y, Yamashita H, Nakamura T, Takahashi T, Kondo E, Nakamura S: Lack of binding observed between human α-synuclein and Bcl-2 protein family. Neurosci Lett 316: 103-107, 2001 Kozuka K, Kohriyama T, Nomura E, Ikeda J, Kajikawa H, Nakamura S：Endothelial markers and adhesion molecules in acute ischemic stroke-sequential change and differences in stroke subtype. Atherosclerosis 161: 161-168, 2002 Nakamura T, Yamashita H, Nagano Y, Takahashi T, Avraham S, Avraham H, Matsumoto M, Nakamura S: Activation of Pyk2/RAFTK induces tyrosine phosphorylation of alpha-synuclein via Src-family kinases. FEBS Lett 521: 190-194, 2002 Naka H, Imon Y, Ohshita T, Honjo K, Kitamura T, Mimori Y, Nakamura S: Magnetization transfer measurements of cerebral white matter in patients with myotonic dystrophy. J Neurol Sci 193: 111-116, 2002 Ogura H, Yasuda M, Nakamura S, Yamashita H, Mikoshiba K, Ohmori H: Neurotoxic damage of granule cells in the dentate gyrus and the cerebellum and cognitive deficit following neonatal administration of phenytoin in mice. J Neuropathol Exp Neurol 61: 956-967, 2002 Maruyama H, Izumi Y, Morino H, Oda M, Toji H, Nakamura S, Kawakami H: Difference in disease-free survival curve and regional distribution according to subtype of spinocerebellar ataxia: A study of 1,286 Japanese patients. Am J Med Genet 114: 578-583, 2002 Inoue K, Yamashita H, Harada T, Nakamura S: Role of human cortices in sensorimotor integration. Clin Neurophysiol 113: 1573-1578, 2002 Shoji M, Matsubara E, Murakami T, Manabe Y, Abe K, Kanai M, Ikeda M, Tomidokoro Y, Shizuka M, Watanabe M, Amari M, Ishiguro K, Kawarabayashi T, Harigaya Y, Okamoto K, Nishimura T, Nakamura Y, Takeda M, Urakami K, Adachi Y, Nakashima K, Arai H, Sasaki H, Kanemaru K, Yamanouchi H, Yoshida Y, Ichise K, Tanaka K, Hamamoto M, Toji H, Nakmura S, Hirai S: Cerebrospinal fluid tau in dementia disorders: a large scale multicenter study by a Japanese study group. Neurobiol Aging 23: 363-370, 2002 Yukawa M, Miyachi T, Ochi H, Katayama S, Kohriyama T, Mimori Y, Nakamura S: Characteristic features of folate-deficient neurological diseases in the elderly. Geriatr Gerontol Int 2: 97-104, 2002 Harada H, Mitsuoka T, Kumagai R, Murata Y, Kaseda Y, Kamei H, Ishizaki F, Nakamura S: Clinical features of malignant syndrome in Parkinson’s disease and related neurological disorders. Parkinsonism Relat Disord 9: S15-S23, 2003 二宮　治明　（鳥取大学・医学部） Ohara S, Ukita Y, Ninomiya H, Ohno K: Degeneration of cholecystokinin-immunoreactive afferents to the VPL thalamus of the mouse model of Niemann-Pick type C. Brain Res 1022: 244-246, 2004 Hou L, Sugimoto Y, Ohsaki Y, Ninomiya H, Oka A, Taniguchi M, Ida H, Eto Y, Ogawa S, Matsuzaki Y, Sawa M, Inoue T, Higaki K, Nanba E, Ohno K, Suzuki Y: N-Octyl-beta-valienamine up-regulates activity of F213I mutant beta-glucosidase in cultured cells: a potential chemical chaperone therapy for Gaucher disease. Biochim Biophys Acta 1689: 219-228, 2004 Kato Y, Akai A, Suzuki R, Hosokawa H, Ninomiya H, Masaki T, Nagata K, Tanokura M: 1H, 13C and 15N assignments of the tandem WW domains of human MAGI-1/BAP-1. J Biomol NMR 29: 539-540, 2004 Wakutani Y, Watanabe K, Adachi Y, Wada-Isoe K, Urakami K, Ninomiya H, Saido TC, Hashimoto T, Iwatsubo T, Nakashima K: Novel amyloid precursor protein gene missense mutation (D678N) in probable familial Alzheimer’s disease. J Neurol Neurosurg Psychiat 75: 1039-1042, 2004 Ohara S, Ukita Y, Ninomiya H, Ohno K: Axonal dystrophy of dorsal root ganglion sensory neurons in a mouse model of Niemann-Pick disease type C. Exp Neurol 187: 289-298, 2004 Ohsaki Y, Sugimoto Y, Suzuki M, Kaidoh T, Shimada Y, Ohno-Iwashita Y, Davies J.P., Ioannou YA, Ohno K, Ninomiya H: Reduced sensitivity of Niemann-Pick C1-deficient cells to theta-toxin (perfringolysin O): sequestration of toxin to raft-enriched membrane vesicles. Histochem Cell Biol 121: 263-272, 2004 Yamamoto T, Feng J.H., Higaki K, Taniguchi M, Nanba E, Ninomiya H, Ohno K: Increased NPC1 mRNA in skin fibroblasts from Niemann-Pick disease type C patients. Brain Dev 26: 245-250, 2004 Feng J.H., Yamamoto T, Nanba E, Ninomiya H, Oka A, Ohno K: Novel TSC2 mutations and decreased expression of tuberin in cultured tumor cells with an insertion mutation. Hum Mutat 24: 397, 2004 Kawashima Y, Kanzaki S, Yang F, Kinoshita T, Hanaki K, Nagaishi J.I., Ohtsuka Y, Hisatome I, Ninomoya H, Nanba E, Fukushima T, Takahashi SI: Mutation at the cleavage site of IGF receptor in short stature children born with intrauterine growth retardation. J Clin Endocrinol Metab 90: 4679-4687, 2005 Sonoyama K, Igawa O, Miake J, Yamamoto Y, Sugihara S, Sasaki N, Shimoyama M, Hamada T, Taniguchi S, Yoshida A, Ogino K, Shigemasa C, Kurata Y, Shiota G, Narahashi T, Horiuchi M, Matsubara H, Ninomiya H, Hisatome I: Effects of angiotensin II on the action potential durations of atrial myocytes in hypertensive rats. Hypertens Res 28: 173-179, 2005 Tanaka H, Miake J, Notsu T, Sonyama K, Sasaki N, IItsuka K, Kato M, Taniguchi S, Igawa O, Yoshida A, Shigemasa C, Hoshikawa H, Kurata Y, Kuniyasu A, Nakayama H, Inagaki N, Nanba E, Shiota G, Morisaki T, Ninomiya H, Kitakaze M, Hisatome I: Proteasomal degradation of Kir6.2 channel protein and its inhibition by a Na+ channel blocker aprindine. Biochem Biophys Res Commun 331: 1001-1006, 2005 Harada M, Kawaguchi T, Kumemura H, Terada K, Ninomiya H, Taniguchi E, Hanada S, Maeyama M, Koga H, Ueno T, Furuta K, Suganuma T, Sugiyama T, Sata M: The Wilson disease protein ATP7B resides in the late endosomes with Rab7 and the Niemann-Pick C1 protein. Am J Pathol 166: 499-510, 2005 貫名　信行　（理化学研究所・脳科学総合研究センター） Nagao Y, Ishiguro H, Nukina N: DMSO and glycerol reduce bacterial death induced by expression of truncated N-terminal huntingtin with expanded polyglutamine tracts. Biochim Biophys Acta 1502: 247-256, 2000 Jana NR, Tanaka M, Wang G, Nukina N: Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity. Hum Mol Genet 9: 2009-2018, 2000 Wang G, Sawai N, Kotliarova S, Kanazawa I, Nukina N: Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B. Hum Mol Genet 9: 1795-1803, 2000 Tanaka M, Morishima I, Akagi T, Hashikawa T, Nukina N: Intra- and intermolecular beta-pleated sheet formation in glutamine-repeat inserted myoglobin as a model for polyglutamine diseases. J Biol Chem 276: 45470-45475, 2001 Jana NR, Zemskov EA, Wang G, Nukina N: Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release. Hum Mol Genet 10: 1049-1059, 2001 Mitsui K, Nakayama H, Akagi T, Nekooki M, Ohtawa K, Takio K, Hashikawa T, Nukina N: Purification of polyglutamine aggregates and identification of elongation factor-1alpha and heat shock protein 84 as aggregate-interacting proteins. J Neurosci 22: 9267-9277, 2002 Tanaka M, Machida Y, Nishikawa Y, Akagi T, Morishima I, Hashikawa T, Fujisawa T, Nukina N: The effects of aggregation-inducing motifs on amyloid formation of model proteins related to neurodegenerative diseases. Biochemistry 41: 10277-10286, 2002 Zemskov EA, Nukina N: Impaired degradation of PKCalpha by proteasome in a cellular model of Huntington's disease. NeuroReport 14: 1435-8, 2003 Wen FC, Li YH, Tsai HF, Lin CH, Li C, Liu CS, Lii CK, Nukina N, Hsieh M: Down-regulation of heat shock protein 27 in neuronal cells and non-neuronal cells expressing mutant ataxin-3. FEBS Lett 546: 307-314, 2003 Tanaka M, Machida Y, Nishikawa Y, Akagi T, Hashikawa T, Fujisawa T, Nukina N: Expansion of polyglutamine induces the formation of quasi-aggregate in the early stage of protein fibrillization. J Biol Chem 278: 34717-34724, 2003 Lee J.A., Lim C.S., Lee S.H., Kim H, Nukina N, Kaang BK: Aggregate formation and the impairment of long-term synaptic facilitation by ectopic expression of mutant huntingtin in Aplysia neurons. J Neurochem 85: 160-169, 2003 Zemskov EA, Jana NR, Kurosawa M, Miyazaki H, Sakamoto N, Nekooki M, Nukina N: Pro-apoptotic protein kinase C delta is associated with intranuclear inclusions in a transgenic model of Huntington’s disease. J Neurochem 87: 395-406, 2003 Jana NR, Dikshit P, Goswami A, Nukina N: Inhibition of proteasomal function by curcumin induces apoptosis through mitochondrial pathway. J Biol Chem 279: 11680-11685, 2004 Tanaka M, Machida Y, Niu S, Ikeda T, Jana NR, Doi H, Kurosawa M, Nekooki M, Nukina N: Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease. Nat Med 10: 148-154, 2004 Venkatraman P, Wetzel R, Tanaka M, Nukina N, Goldberg AL: Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins. Mol Cell 14: 95-104, 2004 Khan LA, Nukina N: Molecular and functional analysis of Caenorhabditis elegans CHIP, a homologue of mammalian CHIP. FEBS Lett 565: 11-18, 2004 Doi H, Mitsui K, Kurosawa M, Machida Y, Kuroiwa Y, Nukina N: Identification of ubiquitin-interacting proteins in purified polyglutamine aggregates. FEBS Lett 571: 171-176, 2004 Nagaoka U, Kim K, Jana N.R., Doi H, Maruyama M, Mitsui K, Oyama F, Nukina N: Increased expression of p62 in expanded polyglutamine-expressing cells and its association with polyglutamine inclusions. J Neurochem 91: 57-68, 2004 Jana NR, Nukina N: Misfolding promotes the ubiquitination of polyglutamine-expanded ataxin-3, the defective gene product in SCA3/MJD. Neurotox Res 6: 523-533, 2004 Jana NR, Dikshit P, Goswami A, Kotliarova S, Murata S, Tanaka K, Nukina N: Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes. J Biol Chem 280: 11635-11640, 2005 Jana NR, Nukina N: BAG-1 associates with the polyglutamine-expanded huntingtin aggregates. Neurosci Lett 378: 171-175, 2005 Kotliarova S, Jana NR, Sakamoto N, Kurosawa M, Miyazaki H, Nekooki M, Doi H, Machida Y, Wong HK, Suzuki T, Uchikawa C, Kotliarov Y, Uchida K, Nagao Y, Nagaoka U, Tamaoka A, Oyanagi K, Oyama F, Nukina N: Decreased expression of hypothalamic neuropeptides in Huntington disease transgenic mice with expanded polyglutamine-EGFP fluorescent aggregates. J Neurochem 93: 641-653, 2005 長谷川　一子　（国立病院機構相模原病院・臨床研究センター） Funayama M, Hasegawa K, Kowa H, Saito M, Tsuji S, Obata F: A new locus for Parkinson’s disease (PARK 8) maps to chromosome 12p11.2-q13.1. Ann Neurol 51: 296-301, 2002 Funayama M, Hasegawa K, Ohta E, Kawashima N, Komiyama M, Kowa H, Tsuji S, Obata F: An LRRK2 mutation as cause for the Parkinsonism in the original PARK 8 family. Ann Neurol 57: 918-921, 2005 服部　信孝　（順天堂大学・医学部） Shimura H, Hattori N, Kubo S, Mizuno Y, Asakawa S, Minoshima S, Shimizu N, Iwai K, Chiba T, Tanaka K, Suzuki T: Familial Parkinson disease gene product, parkin, is a ubiquitin-protein ligase. Nat Genet. 25: 302-305, 2000 Zhang J, Hattori N, Giladi N, Mizuno Y: Failure to find mutations in ubiquitin carboxy-terminal hydrolase L1 gene in familial Parkinson's disease. Parkinsonism Relat Disord 6: 199-200, 2000 Zhang J, Hattori N, Leroy E, Morris HR, Kubo S, Kobayashi T, Wood NW, Polymeropoulos MH, Mizuno Y: Association between a polymorphism of ubiquitin carboxy-terminal hydrolase L1 (UCH-L1) gene and sporadic Parkinson's disease. Parkinsonism Relat Disord 6: 195-197, 2000 Kobayashi T, Wang M, Hattori N, Matsumine H, Kondo T, Mizuno Y: Exonic deletion mutations of the Parkin gene among sporadic patients with Parkinson’s disease. Parkinsonism Relat Disord 6: 129-131, 2000 Shimura H, Hattori N, Kubo S, Mizuno Y, Asakawa S, Minoshima S, Shimizu N, Iwai K, Chiba T, Tanaka K, Suzuki T: Familial parkinson’s disease gene product, Parkin, is a ubiquitin-protein ligase. Nat Genet 25: 302-305, 2000 Urabe T, Yamasaki Y, Hattori N, Yoshikawa M, Uchida K, Mizuno Y: Accumulation of 4-hydroxynonenal-modified proteins in hippocampal CA1 pyramidal neurons precedes delayed neuronal damage in the gerbil brain. Neuroscience 14: 241-250, 2000 Kitada T, Asakawa S, Matsumine H, Hattori N, Shimura H, Minoshima S, Shimizu N, Mizuno Y: Progress in the clinical and molecular genetics of familial parkinsonism. Neurogenetics 2: 207-218, 2000 Shimizu N, Asakawa S, Minoshima S, Kitada T, Hattori N, Matsumine H, Yokochi M, Yamamura Y, Mizuno Y: PARKIN as a pathogemic gene for autosomal recessive juvenile parkinsonism. J Neural Transm [Suppl] 58: 19-30, 2000 Hattori N, Shimura H, Kubo S, Wwang M, Shimizu N, Tanaka K, Mizuno Y: Importance of familial Parkinson’s disease and parkinsonism to the understanding of nigral degeneration in sporadic Parkinson’s disease. J Neural Transm [Suppl] 60: 85-100, 2000 Yamamura Y, Hattori N, Matsumine H, Kuzuhara S, Mizuno Y: Autosomal recessive early-onset parkinsonism with diurnal fluctuation: clinicopathological characteristics and molecular genetic identification. Brain Dev [Suppl] 22: 87-91, 2000 Takanashi M, Mochizuki H, Yokomizo K, Hattori N, Mori H, Yamamura Y, Mizuno Y: Iron accumulation in the substantia nigra of autosomal recessive juvenile parkinsonism (ARJP). Parkinsonism Relat Disord 7: 311-314, 2001 Mellick GD, Buchanan DD, Hattori N, Brookes AJ, Mizuno Y, Le Couteur DG, Silburn PA: The parkin gene S/N167 polymorphism in Australian Parkinson's disease patients and controls. Parkinsonism Relat Disord 7: 89-91, 2001 Lu CS, Wu JC, Tsai CH, Chen RS, Chou YH, Hattori N, Yoshino H, Mizuno Y: Clinical and genetic studies on familial parkinsonism: the first report on a parkin gene mutation in a Taiwanese family. Mov Disord 16: 164-166, 2001 Kubo SI, Kitami T, Noda S, Shimura H, Uchiyama Y, Asakawa S, Minoshima S, Shimizu N, Mizuno Y, Hattori N: Parkin is associated with cellular vesicles. J Neurochem 78: 42-54, 2001 Wang M, Suzuki T, Kitada T, Asakawa S, Minoshima S, Shimizu N, Tanaka K, Mizuno Y, Hattori N: Developmental changes in the expression of parkin and UbcR7, a parkin-interacting and ubiquitin-conjugating enzyme, in rat brain. J Neurochem 77: 1561-1568, 2001 Nisipeanu P, Inzelberg R, Abo Mouch S, Carasso RL, Blumen SC, Zhang J, Matsumine H, Hattori N, Mizuno Y: Parkin gene causing benign autosomal recessive juvenile parkinsonism. Neurology 56: 1573-1575, 2001 Shimura H, Schlossmacher MG, Hattori N, Frosch MP, Trockenbacher A, Schneider R, Mizuno Y, Kosik KS, Selkoe DJ: Ubiquitination of a new form of alpha-synuclein by parkin from human brain: implications for Parkinson's disease. Science 293: 263-269, 2001 Imai Y, Soda M, Inoue H, Hattori N, Mizuno Y, Takahashi R: An unfolded putative transmembrane polypeptide, which can lead to endoplasmic reticulum stress, is a substrate of Parkin. Cell 105: 891-902, 2001 Shimo-Nakanishi Y, Urabe T, Hattori N, Watanabe Y, Nagao T, Yokochi M, Hamamoto M, Mizuno Y: Polymorphism of the lipoprotein lipase gene and risk of atherothrombotic cerebral infarction in the Japanese. Stroke 32: 1481-1486, 2001 Jeon BS, Kim JM, Lee DS, Hattori N, Mizuno Y: An apparently sporadic case with parkin gene mutation in a Korean woman. Arch Neurol 58: 988-989, 2001 Tanaka K, Suzuki T, Chiba T, Shimura H, Hattori N, Mizuno Y: Parkin is linked to the ubiquitin pathway. J Mol Med 79: 482-494, 2001 久永　真市　（東京都立大学・理学研究科） Kitazawa H, Iida J, Uchida A, Haino-Fukushima K, Itoh TJ, Hotani H, Ookata K, Murofushi H, Bulinski JC, Kishimoto T, Hisanaga S: Ser787 in the proline-rich region of human MAP4 is a critical phosphorylation site that reduces the microtubule polymerization activity. Cell Struct Funct 25: 33-39, 2000 Tokuoka H, Saito T, Yorifuji H, Kishimoto T, Hisanaga S: BDNF-induced phosphorylation of neurofilament-H subunit in primary culture of rat cortical neurons. J Cell Sci 113: 1059-1068, 2000 Kusakawa G, Saito T, Onuki R, Ishiguro K, Kishimoto T, Hisanaga S: Calpain-dependent proteolytic cleavage of the p35 CDK5 activtor to p25. J Biol Chem 275: 17166-17172, 2000 Taniguchi S, Fujita Y, Takahashi H, Hayashi S, Kakita A, Murayama S, Saido T, Hisanaga S, Iwatsubo T, Hasegawa M: Calpain-mediated degradation of p35 to p25 in postmortem human and rat brains. FEBS Lett 489: 46-50, 2001 Uchida A, Komiya Y, Tashiro T, Yorifuji H, Kishimoto T, Nabeshima Y, Hisanaga S: The neurofilament of Klotho, the mutant mouse prepaturely displaying symptoms resembling human aging. J Neurosci Res 64: 364-370, 2001 Bibb JA, Nishi A, O’Callaghan JP, Ule J, Lan M, Snyder GL, Horiuchi A, Saito T, Hisanaga S, Czernikj AJ, Nairn AC, Greengard P: Phosphorylation of protein phosphatase inhibitore-I by Cdk5. J Biol Chem 276: 14490-14497, 2001. Chang W, Gruber D, Chari S, Kitazawa H, Hamazumi Y, Hisanaga S, Bulinski JC: Phosphorylation of MAP4 affects microtubule properties and cell cycle progression. J Cell Sci 114: 2879-2887, 2001 Alim MA, Hossain MS, Arima K, Takeda K, Izumiyama Y, Nakamura M, Kaji H, Shinoda T, Hisanaga S, Ueda K: Tubulin seeds α-synuclein fibril formation. J Biol Chem 272: 2112-2117, 2002 Iida J, Itoh TJ, Hotani H, Nishiyama K, Murofushi H, Bulinski JC, Hisanaga S: The projection domain of MAP4 suppresses the microtubule-bundling activity of the microtubule-binding domain. J Mol Biol 320: 97-106, 2002 Sasaki T, Taoka M, Ishiguro K, Uchida A, Saito T, Isobe T, Hisanaga S: In vivo and in vitro phosphorylation at Ser493 in the E-segment of neurofilament-H subunit by GSK3β. J Biol Chem 277: 36032-36039, 2002 Hashiguchi M, Saito T, Hisanaga S, Hashiguchi T: Truncation of CDK5 activator p35 induces intensive phosphorylation of Ser202/Thr205 of human tau 40. J Biol Chem 277: 44524-44530, 2002 Saito T, Onuki R, Fujita Y, Kusakawa G, Ishiguro K, Bibb JA, Kishimoto T, Hisanaga S: Developmental regulation of the proteolysis of the p35 Cdk5 activator by phosphorylation. J Neurosci 23: 1189-1197, 2003 Takahashi S, Saito T, Hisanaga S, Pant HC, Kulkarni AB: Tau phosphorylation by cyclin-dependent kinase 5/p39 during brain development reduces its affinity for microtubules. J Biol Chem 278: 10506-10515, 2003 Kawachi A, Ichihara K, Hisanaga S, Iida J, Toyota H, Hotani H, Itoh TJ: Different protofilament-dependence of the microtubule binding between MAP2 and MAP4. Biochem Biophys Res Commun 305: 72-78, 2003 Honma N, Asada A, Takeshita S, Enomoto M, Yamakawa E, Tsutsumi K, Saito T, Satoh T, Itoh H, Kaziro Y, Kishimoto T, Hisanaga S: Apoptosis-associated tyrosine kinase (AATYK) is a Cdk5 activator p35 binding protein. Biochem Biophys Res Commun 310: 398-404, 2003 Tomizawa K, Sunada S, Lu Y-F, Oda Y, Kinuta M, Ohshima T, Saito T, Matsushita M, Li S-T, Moriwaki A, Tsutsui K, Hisanaga S, Mikoshiba K, Takei K, Matsui H: Cdk5/p35-dependent phosphorylation of amphiphysin I and dynamin I: Critical role in clathrin-mediated endocytosis of synaptic vesicles. J Cell Biol 163: 813-824, 2003 Uchida A, Tashiro T, Komiya Y, Yorifuji H, Kishimoto T, Hisanaga S: Morphological and biochemical changes of neurofilaments in aged rat sciatic nerve axons. J Neurochem 88: 735-745, 2004 Alim MA, Ma Q-L, Takeda K, Aizawa T, Matsubara M, Nakamura M, Saito T, Asada A, Kaji H, Yoshii M, Hisanaga S, Ueda K: Demonstration of a role for alpha-synuclein as a functional microtubule-associated protein. J Alz Dis 6: 435-442, 2004 Hatanaka Y, Hisanaga S, Heizmann CW, Murakami F: Distinct migratory behavior of early-and late-born neurons in the cerebral cortex. J Comp Neurol 479: 1-14, 2004 Wei F-Y, Tomizawa K, Ohshima T, Asada A, Saito T, Nguyen C, Bibb JA, Ishiguro K, Kulkarni AB, Pant HC, Mikoshiba K, Matsui H, Hisanaga S: Control of cyclin-dependent kinase 5 (Cdk5) activity by glutamatergic regulation of p35 stability. J Neurochem 93: 502-512, 2005 Permana S, Hisanaga S, Nagatomo Y, Iida J, Hotani H, Itoh TJ: Truncation of the projection domain of MAP4 (microtubule-associated protein 4) leads to the attenuation of dynamic instability of microtubules. Cell Struct Funct 29: 147-157, 2005 Taniguchi S, Suzuki N, Masuda M, Hisanaga S, Iwatsubo T, Goedert M, Hasegawa M: Inhibition of heparin-induced tau filament formation by phenotiazine, polyphenols and porphyrins. J Biol Chem 280: 7614-7623, 2005 Ohshima T, Ogura H, Tomizawa K, Hayashi K, Suzuki H, Saito T, Kamei H, Nishi A, Bibb JA, Hisanaga S, Matsui H, Mikoshiba K: Impairment of hippocampal long-term depression and defective spatial learning and memory in p35 mice. J Neurochem 94: 917-925, 2005 Zhu Y-S, Saito T, Asada A, Maekawa S, Hisanaga S: Activation of latent cyclin-dependent kinase 5 (Cdk5)-p35 complexes by membrane dissociation. J Neurochem 94: 1535-1545, 2005 船越　洋　（大阪大学・大学院医学系研究科） Isogawa K, Akiyoshi J, Kodama K, Matsushita H, Tsutsumi T, Funakoshi H, Nakamura T: Anxiolytic effect of hepatocyte growth factor infused into rat brain. Neuropsychobiology 51: 34-38, 2004 Chiyonobu T, Sasaki J, Nagai Y, Takeda S, Funakoshi H, Nakamura T, Sugimoto T, Toda T: Effects of fukutin deficiency in the developing mouse brain. Neuromuscul Disord 15: 416-426, 2005 堀江　秀典　（早稲田大学・先端バイオ研究所） Inagaki Y, Sohma Y, Horie H, Nozawa R, Kadoya T: Oxidized galectin-1 promotes axonal regeneration in peripheral nerves but does not possess lectin properties. Eur J Biochem 267: 2955-2964, 2000 Horie H, Kadoya T: Identification of oxidized galectin-1 as an initial repair regulatory factor after axotomy in peripheral nerves. Neurosci Res 38: 131-137, 2000 Shuto T, Horie H, Hikawa N, Sango K, Tokashiki A, Murata H, Yamamoto I, Ishikawa Y: IL-6 upregulates CNTF mRNA expression and enhances neurite regeneration. NeuroReport 12: 1081-1085, 2001 Kato T, Kurita K, Seino T, Kadoya T, Horie H, Wada M, Kawanami T, Daimon M, Hirano A: Galectin-1 is a component of neurofilamentous lesions in sporadic and familial amyotrophic lateral sclerosis. Biochem Biophys Res Commun 282: 166-172, 2001 Hirono M, Sugiyama T, Kishimoto Y, Sakai I, Miyazawa T, Kishio M, Inoue H, Nakao K, Ikeda M, Kawahara S, Kirino Y, Katsuki M, Horie H, Ishikawa Y, Yoshioka T: Phospholipase Cβ4 and protein kinase Cα and/or protein kinase CβI are involved in the induction of long-term depression in cerebellar Purkinje cells. J Biol Chem 276: 45236-45242, 2001 Takano M, Horie H, Iijima Y, Dezawa M, Sawada H, Ishikawa Y: Brain-derived neurotrophic factor enhances neurite regeneration from retinal ganglion cells in aged human retina in vitro. Exp Eye Res 74: 319-323, 2002 Saito H, Sango K, Horie H, Takeshita K, Ikeda H, Ishigatsubo Y, Ishikawa Y: Trachea enhances neurite regeneration from adult rat nodose ganglia in vitro. Life Sci 70: 1935-1946, 2002 Nishioka T, Sakumi K, Miura T, Tahara K, Horie H, Kadoya T, Nakabeppu Y: FosB gene products trigger cell proliferation and morphlogical alteration with an increased expression of a novel processed form of galectin-1 in the rat 3Y1 embryo cell line. J Biochem 131: 653-661, 2002 Oshitari T, Dezawa M, Okada S, Takano M, Negishi h, Horie H, Sawada H, Tokuhisa T, Adachi-Usami E: Suppression of c-fos mediates inhibition of cell death and increases neurite regeneration in retinal ganglion cells. Invest Ophthalmol Vis Sci 43: 2442-2449, 2002 Sango K, Horie H, Saito H, Ajiki K, Tokashiki A, Takeshita K, Ishigatsubo Y, Kawano H, Ishikawa Y: Diabetes is not a potent inducer of neuronal cell death in mouse sensory ganglia, but it enhances neurite regeneration in vitro. Life Sci 71: 2351- 2368, 2002 Fukaya K, Hasegawa M, Kadoya T, Horie H, Fujisawa H, Hayashi Y, Tachibana O, Kida S, Yamashita J: Oxidized galectin-1 stimulates the migration of Schwann cells from both proximal and distal stumps of transected nerves and promotes axonal regeneration after peripheral nerve injury. J Neuropathol Exp Neurrol 62: 162-172, 2003 Tahara K, Tsuchimoto D, Tominaga Y, Asoh S, Ohta S, Kitagawa M, Horie H, Kadoya T, Nakabeppu Y: ΔFosB induces the delayed apoptosis independent of cell proliferation of the Rat1a embryo cell line. Cell Death Differ 10: 496-507, 2003 Imbe H, Okamoto K, Kadoya T, Horie H, Senba E: Galectin-1 is involved in the potentiation of neuropathic pain in the dorsal horn. Brain Res 993: 72-83, 2003 Horie H, Kadoya T: Galectin-1 plays essential roles in adult mammalian nervous tissues. -Roles of oxidized galectin-1. Glycoconj J 19: 479-489, 2004 Sango K, Tokashiki A, Ajiki K, Horie M, Kawano H, Watabe K, Horie H, Kadoya T: Synthesis, localization and externalization of galectin-1 in mature dorsal root ganglion neurons and Schwann cells. Eur J Neurosci 19: 55-64, 2004 Horie H, Kadoya T, Hikawa N, Sango K, Inoue H, Takeshita K, Asawa R, Hiroi T, Sato M, Yoshioka T, Ishikawa Y: Oxidized galectin-1 stimulates macrophages to promote axonal regeneration in peripheral nerves after axotomy. J Neurosci 24: 1673-1680, 2004 Akazawa C, Nakamura Y, Sango K, Horie H, Kohsaka S: Distribution of the galectin-1 mRNA in the rat nervous system; itstransient up-regulation in rat facial motor neurons after facial nerve axotomy. Neuroscience125: 171-178, 2004 Miura T, Takahashi M, Horie H, Tsuchimoto D, Sakumi K, Nakabeppu Y: Galectin-1, a natural monomeric form of galectin-1 lacking its six amino-terminal residues promotes axonal regeneration but not cell death. Cell Death Differ 11: 1076-1083, 2004 McGraw J, Oschipok LW, Liu J, Hiebert GW, Mak CFW, Horie H, Kadoya T, Steeves JD, Ramer MS, Tetzlaff W: Galectin-1 expression correlates with the regenerative potential of rubrospinal and spinal motoneurons. Neuroscience 128: 713-719, 2004 Tsukiji J, Sango K, Udaka N, Kageyama H, Ito T, Saito H, Horie H, Inoue S, Kitamura H, Hagiwara E, Ikeda H, Okubo T, Ishigatsubo Y: Long term induction of β-CGRP mRNA in rat lungs by allergic inflammation. Life Sci 76: 163-77, 2004 McGraw J, McPhail LT, Oschipok LW, Horie H, Poirier F, Steeves JD, Ramer MS, Tetzlaff W: Galectin-1 in regenerating motoneurons. Eur J Neurosci 20: 2872-2880, 2004 松井　秀樹　（岡山大学・大学院医歯学総合研究科） Tomizawa K, Cai XH, Moriwaki A, Matsushita M, Matsui H: Involvement of cyclin-dependent kinase 5/p35(nck5a) in the synaptic reorganization of rat hippocampus during kindling progression. Jpn J Physiol 50: 525-532, 2000 Matsushita M, Tomizawa K, Moriwaki A, Li ST, Terada H, Matsui H: A high-efficiency protein transduction system demonstrating the role of PKA in long-lasting long-term potentiation. J Neurosci 21: 6000-6007, 2001 Kurazono S, Okamoto M, Mori S, Matsui H: Recombinant core proteuin fragment of phosphacan, a brain specific chondroitin sulfate proteoglycan, promote excitotoxic cell death of cultured rat hippocampal neurons. Neurosci Lett 304: 169-172, 2001 Kurazono S, Okamoto M, Sakiyama J, Mori S, Nakata Y, Fukuoka J, Amano S, Oohira A, Matsui H: Expression of brain specific chondroitin sulfate proteoglycans, neurocan and phosphacan, in the developing and adult hippocampus of Ihara’s epileptic rats. Brain Res 898: 36-48, 2001 Tomizawa K, Ohta J, Matsushita M, Moriwaki A, Li ST, Takei K, Matsui H: Cdk5/p35 regulates neurotransmitter release through phosphorylation and downregulation of P/Q-type voltage-dependent calcium channel activity. J Neurosci 22: 2590-2597, 2002 松山　知弘　（兵庫医科大学・医学部・総合内科） Hirata A, Yoshida S, Inoue N, Matsumoto-Miyai K, Ninomiya A, Taniguchi M, Matsuyama T, Kato K, Iizasa H, Kataoka Y, Yoshida N, Shiosaka S: Abnormalities of synapses and neurons in the hippocampus of neuropsin-deficient mice. Mol Cell Neurosci 17: 600-610, 2001 Tamatani M, Matsuyama T, Yamaguchi A, Mitsuda N, Tsukamoto Y, Taniguchi M, Che YH, Ozawa K, Hori O, Nishimura H, Yamashita A, Okabe M, Yanagi H, Stern DM, Ogawa S, Tohyama M: ORP150 protects against hypoxia/ischemia-induced neuronal death. Nat Med 7: 317-323, 2001 Okamoto M, Nakajima Y, Matsuyama T, Sugita M: Amyloid precursor protein associates independently and cooperatively with PTB and PDZ domains of mint on vesicles and at cell membrane. Neuroscience 104: 653-665, 2001 Nakajima Y, Okamoto M, Nishimura H, Obata K, Kitano H, Sugita M, Matsuyama T: Neuronal expression of mint1 and mint2, novel multimodular proteins, in adult murine brain. Mol Brain Res 92: 27-42, 2001 Kitada O, Kodama T, Kuribayashi K, Ihaku D, Fujita M, Matsuyama T, Sugita M: Heme oxygenase-1 (HO-1) protein induction in a mouse model of asthma. Clin Exp Allergy 31: 1470-1477, 2001 Kuribayashi K, Kodama T, Okamura H, Sugita M, Matsuyama T: Effects of postinhalation treatment with interleukin (IL)-12 on airway hyperreactivity, eosinophilia, and IL-18 receptor expression in mouse model of asthma. Clin Exp Allergy 32: 641-649, 2002 Miyazaki M, Ozawa K, Hori O, Kitao Y, Matsushita K, Ogawa S, Matsuyama T: Expression of 150-kd oxygen-regulated protein in the hippocampus suppresses delayed neuronal cell death. J Cereb Blood Flow Metab 22: 979-987, 2002 Ma Y, Okamoto M, Gu F, Obata K, Matsuyama T, Desaki J, Tanaka J, Sakanaka M: Neuronal distribution of EHSH1/intersectin: Molecular linker between clathrin-mediated endocytosis and signaling pathways. J Neurosci Res 71: 468-477, 2002 丸山　和佳子　（国立療養所中部病院長寿医療研究センター） Uezono T, Matsubara K. Maruyama W, Naoi M, Shimizu K, Saito O, Ogawa K, Mizukami H, Hayase N, Shiono H: Norharman, an indoleamine-derived beta-carboline, but not Trp-p-2, a gamma-carboline, induces apoptotic cell death in human neuroblastoma SH-SY5Y cells. J Neural Transm 108: 943-953, 2001 Toth BE, Homicsko K, Radnai B, Maruyama W, DeMaria JE, Vecsernyes M, Fekete MIK, Fülöp F, Naoi M, Freeman ME, Nagy M: Salsolinol is a putative endogenous neuro-intermediate lobe prolactin releasing factor. J Neuroendocrinol 13: 1-14, 2001 Maruyama W, Akao Y, Youdim MBH, Davis BA, Naoi M: Transfection-enforced Bcl-2 overexpression and an anti-Parkinson drug, rasagiline, prevent nuclear accumulation of glyceraldehyde-3-phosphate dehydrogenase induced by an endogenous dopaminergic neurotoxin, N-methyl(R)salsolinol. J Neurochem 78: 727-735, 2001 Maruyama W, Youdim MB, Naoi M: Antiapoptotic proparties of rasagiline, N-propargylamine- 1(R)-aminoindan, and its optical (S)-isomer. TV1022. Ann NY Acad Sci 939: 320-329, 2001 Maruyama W, Kato Y, Yamamoto T, Oh-hashi K, Hashizume Y, Naoi M: Peroxynitrite induces neuronal cell death in aging and age-associated disorders. J Am Aging Assoc 24: 11-18, 2001 Naoi M, Maruyama W, Akao Y, Zhang J, Parvez H: Apoptosis induced by an endogenous neurotoxin, N-methyl(R)salsolinol, in dopamine neurons. Toxicology 153: 123-141, 2001 Oh-hashi K, Maruyama W, Isobe K: Peroxynitrite induces gadd34, 45 and 153 via p38 MAPK in human neuroblastoma SH-SY5Y cells. Free Radic Biol Med 30: 213-221, 2001 Maruyama W, Boulton AA, Davis BA, Dostert P, Naoi M: Enantio-specific induction of apoptosis by an endogenous neurotoxin, N-methyl(R)salsolinol, in dopaminergic SH-SY5Y cells: Suppression of apoptosis by N-(2-heptyl)-N-methylpropargylamine. J Neural Transm 108: 11-24, 2001 三浦　正幸　（東京大学・大学院薬学系研究科） Mochizuki H, Hayakawa H, Migita M, Shibata M, Tanaka R, Suzuki A, Shimo-Nakanishi Y, Urabe T, Yamada M, Tamayose K, Shimada T, Miura M, Mizuno Y: An AAV-derived Apaf-1 dominant negative inhibitor prevents MPTP toxicity as anti-apoptotic gene therapy for Parkinson’s disease. Proc Natl Acad Sci USA 98: 10918-10923, 2001 Shibata M, Kanamori S, Ohsawa Y, Watanabe T, Yayoi Y, Miura M, Kominami E, Uchiyama Y: Prevention of apoptosis of mammalian cells by the CED-3-cleaved form of CED-9. Arch Histol Cytol 64: 17-28, 2001 Kim KW, Chung HH, Chung CW, Kim IK, Miura M, Wang S, Zhu H, Moon KD, Rha GB, Park JH, Jo DG, Woo HN, Song YH, Kim BJ, Yuan J, Jung YK: Inactivation of farnesyltransferase and geranylgeranyltransferase I by caspase-3：Cleavage of the common alpha subunit during apoptosis. Oncogene 20: 358-366, 2001 Hisahara S, Yuan J, Momoi T, Okano H, Miura M: Caspase-11 mediates oligodendrocytes cell death and pathogenesis of autoimmune-mediated demyelination. J Exp Med 193: 111-122, 2001 Kuranaga E, Kanuka H, Igaki T, Sawamoto K, Ichijo H, Okano H, Miura M: Reaper-mediated inhibition of DIAP1-induced Drosophila TRAF1 degradation leads to JNK activation. Nat Cell Biol 4: 705-710, 2002 Takeyama K, Ito S, Yamamoto A, Tanimoto H, Furutani T, Kanuka H, Miura M, Tabata T, Kato S: Androgen-dependent neurodegeneration by polyglutamine-expanded human androgen receptor in Drosophila. Neuron 35: 855-864, 2002 Kanda H, Igaki T, Kanuka H, Yagi T, Miura M: Wengen, a member of the Drosophila TNF receptor superfamily, is required for Eiger signaling. J Biol Chem 277: 28372-28375, 2002 Igaki T, Kanda H, Yamamoto-Goto Y, Kanuka H, Kuranaga E, Aigaki T, Miura M: Eiger, a TNF superfamily ligand that triggers the Drosophila JNK pathway. EMBO J 21: 3009-3018, 2002 Igaki T, Yamamoto-Goto Y, Tokushige N, Kanda H, Miura M: Downregulation of DIAP1 triggers a novel Drosophila cell death pathway mediated by DARK and DRONC. J Biol Chem 277: 23103-23106, 2002 Shibata M, Yamawaki T, Hattori H, Hamada J, Fukuuchi Y, Okano H, Miura M: Upregulation of Akt phosphorylation at the early stage of middle cerebral artery occlusion in mice. Brain Res 942: 1-10, 2002 Araki T, Hayashi M, Watanabe N, Kanuka H, Yoshino J, Miura M, Saruta T: Down regulation of Mcl-1 by inhibition of the PI3-K/Akt pathway is required for cell shrinkage-dependent cell death. Biochem Biophys Res Commun 290: 1275-1281, 2002 Qiao J, Mitsuhara I, Yazaki Y, Sakano K, Gotoh Y, Miura M, Ohashi Y: Enhanced registance to salt, cold and wound stresses by overproduction of animal cell death suppressors Bcl-xL and Ced-9 in tobacco cells: Their possible contribution through improved function of organella. Plant Cell Physiol 43: 992-1005, 2002 Inoue H, Tsukita K, Iwasato T, Suzuki Y, Tomioka M, Tateno M, Nagao M, Kawata A, Saido TC, Miura M, Misawa H, Itohara S, Takahashi R: The crucial role of caspase-9 in the disease progression of a transgenic ALS mouse model. EMBO J 22: 6665-6674, 2003 Kanuka H, Kuranaga E, Hiratou T, Igaki T, Nelson B, Okano H, Miura M: Cytosol-ER interplay by Sec61α translocon in polyglutamine-mediated neurotoxicity in Drosophila. Proc Natl Acad Sci USA 100: 11723-11728, 2003 Takahashi M, Kanuka H, Fujiwara H, Koyama A, Hasegawa M, Miura M, Iwatsubo T: Phosphorylation of alpha-synuclein characteristic of synucleinopathy lesions is recapitulated in alpha-synuclein transgenic Drosophila.  Neurosci Lett 336: 155-158, 2003 Takemoto K, Nagai T, Miyawaki A, Miura M: Spatio-temporal activation of caspase revealed by indicator that is insensitive to environmental effects. J Cell Biol 160: 235-243, 2003 Arai H, Furuya T, Yasuda T, Miura M, Mizuno Y, Mochizuki H: Neurotoxic effects of lipopolysaccharide on nigral dopaminergic neurons are mediated by microglial activation, interleukin-1β and expression of caspase-11 in mice. J Biol Chem 279: 51647-51653, 2004 Kakinuma Y, Saitoh F, Osawa S, Miura M: A mechanism of impaired mobility of oligodendrocyte progenitor cells by tenascin C through modification of Wnt signaling. FEBS Lett 568: 60-64, 2004 Kakinuma Y, Saitoh F, Ohsawa S, Furuichi T, Miura M: A sulfatase regulating the migratory potency of oligodendrocyte progenitor cells through tyrosine phosphorylation of β-catenin. J Neurosci Res 77: 653-661, 2004 Furuya T, Hayakawa H, Yamada M, Yoshimi K, Hisahara S, Miura M, Mizuno Y, Mochizuki H: Caspase-11 mediates inflammatory dopaminergic cell death in the 1-Methyl-4-Phenyl-1, 2, 3, 6-Tetrahydropyridine mouse model of Parkinson's disease. J Neurosci 24: 1865-1872, 2004 Iwawaki T, Akai R, Kohno K, Miura M: A transgenic mouse model for monitoring endoplasmic reticulum stress. Nat Med 10: 98-102, 2004 Ohsawa S, Hamada S, Kakinuma Y, Yagi T, Miura M: A novel function of neuronal PAS domain protein 1 (NPAS1) in erythropoietin expression in neuronal cells. J Neurosci Res 79: 451-458, 2005 Kanuka H, Hiratou T, Igaki T, Kanda H, Kuranaga E, Sawamoto K, Aigaki T, Miura M: Gain-of-function screen identifies a role of the Sec61α translocon in Drosophila postmitotic neurotoxicity. Biochim Biophys Acta in press Senoo-Matsuda N, Igaki T, Miura M: Bax-like protein Drob-1 protects neurons from expanded polyglutamine-induced toxicity in Drosophila.EMBO J 24: 2700-2713, 2005 Tamura M, Nakamura M, Ogawa Y, Toyama Y, Miura M, Okano H: Targeted expression of anti-apoptotic protein p35 in oligodendrocytes reduces delayed demyelination and functional impairment after spinal cord injury. Glia 51: 312-321, 2005 Nelson B, Nishimura S, Kanuka H, Kuranaga E, Inoue M, Hori G, Nakahara H, Miura M: Isolation of gene sets affected specifically by polyglutamine expression: implication of the TOR signaling pathway in neurodegeneraton. Cell Death Differ 12: 1115-1123, 2005 水澤　英洋　（東京医科歯科大学・大学院医歯学総合研究科） Mitoma H, Song SY, Ishida K, Yamakuni T, Kobayashi T, Mizusawa H: Presynapic impairment of cerebellar inhibitory synapses by an autoantibody to glutamate decarboxylase. J Neurol Sci 175: 40-44, 2000 Toru S, Murakoshi T, Ishikawa K, Saegusa H, Fujigasaki H, Uchihara T, Nagayama S, Osanai M, Mizusawa H, Tanabe T: Spinocerebellar ataxia type 6 mutation alters P-type calcium channel function.J Biol Chem275: 10893-10898, 2000 Yang Q, Hashizume Y, Yoshida M, Wang Y, Goto Y, Mitsuma N, Ishikawa K, Mizusawa H: Morphological Purkinje cell changes in spinocerebellar ataxia type 6. Acta Neuropathol 100: 371-376, 2000 Yokota T, Uchihara T, Kumagai J, Shiojiri T, Pang JJ, Arita M, Arai H, Hayashi M, Kiyosawa M, Okeda R, Mizusawa H: Postmortem study of ataxia with retinitis pigmentosa by mutation of the α-tocopherol transfer protein. J Neurol Neurosurg Psychiat 68: 521-525, 2000 Kanda T, Iwasaki T, Yamawaki M, Tai T, Mizasawa H: Anti-GM-1 antibody facilitates leakage in an in vitro blood-nerve barrier model. Neurology 55: 585-587, 2000 Orimo S, Ozawa E, Uematsu M, Yoshida E, Hino H, Yamada M, Okeda R, Mizusawa H: A case of Creutzfeldt-Jakob disease presenting with auditory agnosia as an initial manifestation. Eur Neurol 44: 256-258, 2000 Sasaki J, Ishikawa K, Kobayashi K, Kondo IE, Fukayama M, Mizusawa H, Takashima S, Sakakihara Y, Nakamura Y, Toda T: Neural expression of the fukutin gene. Hum Mol Genet 9: 3083-3090, 2000 Nagaoka U, Takashima M, Ishikawa K, Yoshizawa K, Yoshizawa T, Ishikawa M, Yamawaki T, Shoji S, Mizusawa H: A gene on SCA4 locus causes dominantly inherited pure cerebellar ataxia. Neurology 54: 1971-1975, 2000 Fujigasaki H, Uchihara T, Koyano S, Iwabuchi K, Yagishita S, Makifuchi T, Nakamura A, Ishida K, Toru S, Hirai S, Ishikawa K, Tanabe T, Mizusawa H: Ataxin-3 is translocated into the nucleus for the formation of intranuclear inclusions in normal and Machado-Joseph disease brains. Exp Neurol 165: 248-256, 2000 Kanda T, Iwasaki T, Nakamura S, Kurokawa T, Ikeda K, Mizusawa H: Self-secretion of fibroblast growth factor-9 supports basal forebrain cholinergic neurons in an autocrine/paracrine manner. Brain Res 876: 22-30, 2000 Yaginuma M, Ishida K, Uchihara T, Suzuki F, Aoki M, Tanaka T, Murase H, Ikeda K, Mizusawa H: Paraneoplastic cerebellar ataxia with mild cerebello-olivary degeneration and an anti-neuronal antibody: a clinicopathological study. Neuropathol Appl Neurobiol 26: 568-572, 2000 Takashima M, Ishikawa K, Nagaoka U, Shoji S, Mizusawa H: A linkage disequilibrium at the candidate gene locus for 16q-linked autosomal dominant cerebellar ataxia type III in Japan. J Hum Genet 46: 167-171, 2001 Ishikawa K, Owada K, Ishida K, Fujigasaki H, Shun LM, Tsunemi T, Ohkoshi N, Toru S, Mizutani T, Hayashi M, Arai N, Hasegawa K, Kawanami T, Kato T, Makifuchi T, Shoji S, Tanabe T, Mizusawa H: Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells. Neurology 56: 1753-1756, 2001 Takenoshita H, Shizuka IM, Mitoma H, Song S, Harigaya Y, Igeta Y, Yaguchi M, Ishida K, Shoji M, Tanaka M, Mizusawa H, Okamoto K: Presynaptic inhibition of cerebellar GABAergic transmission by glutamate decarboxylase autoantibodies in progressive cerebellar ataxia. J Neurol Neurosurg Psychiat 70: 386-389, 2001 Fujigasaki H, Uchihara T, Takahashi J, Matsushita H, Nakamura A, Koyano S, Iwabuchi K, Hirai S, Mizusawa H: Preferential recruitment of ataxin-3 independent of expanded polyglutamine: an immunohistochemical study on Marinesco bodies. J Neurol Neurosurg Psychiat 71: 518-520, 2001 Yokota T, Igarashi K, Uchihara T, Jishage K, Tomita H, Inaba A, Li Y, Arita M, Suzuki H , Mizusawa H, Arai H: Delayed-onset ataxia in mice lacking a-tocopherol transfer protein: Model for neuronal degeneration caused by chronic oxidative stress. Proc Natl Acad Sci USA 98: 15185-15190, 2001 Tsunemi T, Saegusa H, Ishikawa K, Nagayama S, Murakoshi T, Mizusawa H, Tanabe T: Novel Cav2.1 splice variants isolated from Purkinje cells do not generate P-type Ca2+ current. J Biol Chem 277: 7214-7221, 2002 Miake H, Mizusawa H, Iwatsubo T, Hasegawa M: Biochemical characterization of the core structure of α synuclein filaments. J Biol Chem 277: 19213-19219, 2002 Ohara S, Iwahashi T, Oide T, Hayashi R, Nakajima T, Ishikawa K, Mizusawa H: Spinocerebellar ataxia type6 with motor neuron loss: A follow-up autopsy report. J Neurol 294: 633-635, 2002 Owada K, Uchihara T, Ishida K, Mizusawa H, Watabiki S, Tsuchiya K: Motor weakness and cerebellar ataxa in Sjogren syndrome- identification of antineuronal antibody: a case report. J Neurol Sci 197: 79-84, 2002 Kubodera T, Yokota T, Ohwada K, Ishikawa K, Miura H, Matsuoka T, Mizusawa H: Proteolytic cleavage and cellular toxicity of the human α1A calcium channel in spinocerebellar ataxia type 6 (SCA6). Neurosci Lett 341: 74-78, 2003 Li M, Ishikawa K, Toru S, Tomimitsu H, Takashima M, Goto J, Takiyama Y, Sasaki H, Imoto I, Imazawa J, Toda T, Kanazawa I, Mizusawa H: Physical map and haplotype analysis of 16q-linked autosomal dominant cerebellar ataxia (ACDA) type III in Japan. J Hum Genet 48: 111-118, 2003 Mitoma H, Ishida K, Ikeda M, Mizusawa H: Dual impariment of GABAA- and GABAB-receptor-mediated synaptic responses by autoantibodies to glutamic acidic decarboxylase. J Neurol Sci 208: 51-56, 2003 Yokota T, Sugawara K, Ito K, Takahashi R, Ariga H, Mizusawa H: Down regulation of DJ-1 enhances cell death by oxidative stress, ER stress, and proteasome inhibition. Biochem Biophys Res Commun 312: 1342-1348, 2003 Takahashi H, Ishikawa K, Tsutsumi T, Fujigasaki H, Kawata A, Okiyama R, Fujita T, Yoshizawa K, Yamaguchi S, Tomiyasu H, Yoshii F, Mitani K, Shimizu N, Yamazaki M, Miyamoto T, Orimo T, Shoji S, Kitamura K, Mizusawa H: A clinical and genetic study in a large cohort of patients with spinocerebellar ataxia type 6. J Hum Genet 49: 256-264, 2004 Li Y, Yokota T, Matsumura R, Taira K, Mizusawa H: Sequence-dependent and independent inhibition specific for mutant ataxin-3 by small interfering RNA. Ann Neurol 56: 124-129, 2004 宮田　雄平　（日本医科大学・医学部） Ui-Tei K, Nagano M, Sato S, Miyata Y: Calmodulin-dependent and -independent apoptosis in cells of a Drosophila neuronal cell line. Apoptosis 5: 133-140, 2000 Ui-Tei K, Zenno S, Miyata Y, Saigo K: Sensitive assay of RNA interference in Drosophila and Chinese hamster cultured cells using firefly luciferase gene as target. FEBS Lett 479: 79-82, 2000 Hamada T, Ui-Tei K, Miyata Y: A novel gene derived from developing spinal cords, SCDGF, is a unique member of the PDGF/VEGF family. FEBS Lett 475: 97-102, 2000 Hamada T, Ui-Tei K, Imaki J, Miyata Y: Molecular cloning of SCDGF-B, a novel growth factor homologous to SCDGF/PDGF-C/ fallotein. Biochem Biophys Res Commun 280: 733-737, 2001 Nagano M, Ui-Tei K, Suzuki H, Piao ZF, Miyata Y: CDK inhibitors suppress apoptosis induced by chemicals and by excessive expression of a cell death gene, reaper, in Drosophila cells. Apoptosis 5: 543-550, 2000 村本　環　（東北大学・大学院医学系研究科） Kitamoto T, Mohri S, Ironside JW, Miyoshi I, Tanaka T, Kitamoto N, Itohara S, Kasai N, Katsuki M, Higuchi J, Muramoto T, Shin R-W: Follicular dendritic cell of the knock-in mouse provides a new bioassay for human prions. Biochem Biophys Res Commun 294: 280-286, 2002 森下　竜一　（大阪大学・大学院医学系研究科） Sakaue G, Shimaoka M, Fukuoka T, Hiroi T, Inoue T, Hashimoto N, Sakaguchi T, Sawa Y, Morishita R, Kiyono H, Noguchi K, Mashimo T: NF-kappaB decoy suppresses cytokine expression and thermal hyperalgesia in a rat neuropathic pain model. NeuroReport 12: 2079-2084, 2001 Hayashi K, Morishita R, Nakagami H, Yoshimura S, Hara A, Matsumoto K, Nakamura T, Kaneda Y, Ogihara T, Sakai N: Gene therapy for preventing neuronal death using hepatocyte growth factor: in vivo gene transfer of HGF to subarachnoid space prevents delayed neuronal death in gerbil hippocampal CA1 neurons. Gene Ther 8: 1167-1173, 2001 Sakaue G, Shimaoka M, Fukuoka T, Hiroi T, Inoue T, Hashimoto N, Sakaguchi T, Sawa Y, Morishita R, Kiyono H, Noguchi K, Mashimo T: NF-kappa B decoy suppresses cytokine expression and thermal hyperalgesia in a rat neuropathic pain model. NeuroReport 12: 2079-2084, 2001 Inoue T, Sawa Y, Kitagawa-Sakakida S, Nishimura M, Morishita R, Kaneda Y, Kohmura E, Yoshimine T, Matsuda H: Nuclear factor-kappaB decoy attenuates neuronal damage after global brain ischemia: A future strategy for brain protection during circulatory arrest. J Thorac Cardiovasc Surg 122: 720-727, 2001 Yoshimura S, Morishita R, Hayashi K, Yamamoto K, Nakagami H, Kaneda Y, Sakai N, Ogihara T: Inhibition of intimal hyperplasia after balloon injury in rat carotid artery model using cis-element "decoy" of nuclear factor-kB binding site as a novel molecular strategy. Gene Ther 8: 1635-1642, 2001 Taniyama Y, Tachibana K, Hiraoka K, Namba T, Yamasaki K, Hashiya N, Aoki M, Ogihara T, Kaneda Y, Morishita R: Local delivery of plasmid DNA into rat carotid artery using ultrasound. Circulation 105: 1233-1239, 2002 Mori M, Nakagami H, Morishita R, Mitsuda N, Yamamoto K, Yoshimura S, Ohkubo N, Sato N, Ogihara T, Kaneda Y: N1411 mutant presenilin-2 gene enhances neuronal cell death and decreases bcl-2 expression. Life Sci 70: 2567-2580, 2002 Yoshimura S, Morishita R, Hayashi K, Kokuzawa J, Aoki M, Matsumoto K, Nakamura T, Ogihara T, Kaneda Y, Sakai N: Gene transfer of hepatocyte growth factor to subarachnoid space in cerebral hypoperfusion model. Hypertension 39: 1028-1034, 2002 Shimamura M, Morishita R, Endoh M, Oshima K, Aoki M, Waguri S, Uchiyama Y, Kaneda Y: HVJ-envelope vector for gene transfer into central nervous system. Biochem Biophys Res Commun 300: 464-471, 2003 矢澤　生　（沖中記念成人病研究所） Hazeki N, Tsukamoto T, Yazawa I, Koyama M, Hattori S, Someki I, Iwatsubo T, Nakamura K, Goto J, Kanazawa I: Ultrastructure of nuclear aggregates formed by expressing an expanded polyglutamine. Biochem Biophys Res Commun 294: 429-440, 2002 Yazawa I, Hazeki N, Nakase H, Kanazawa I, Tanaka M: Histone H3 is aberrantly phosphorylated in glutamine-repeat diseases. Biochem Biophys Res Commun 302: 144-149, 2003 柳　茂　（神戸大学・大学院医学系研究科） Takahashi S, Inatome R, Hotta A, Qin Q, Hackenmiller R, Simon M.C, Yamamura H, Yanagi S: Role for Fes/Fps tyrosine kinase in microtubule nucleation through its FCH domain. J Biol Chem 278: 49129-49133, 2003 Hirose M, Kitano J, Nakajima Y, Moriyoshi K, Yanagi S, Yamamura H, Muto T, Jingami H, Nakanishi S: Phosphorylation and recruitment of Syk by ITAM-based phosphorylation of tamalin. J Biol Chem 279: 32308-32315, 2004 Hotta A, Inatome R, Yuasa-Kawada J, Qin Q, Yamamura H, Yanagi S: Critical role of CRMP-associated molecule CRAM for filopodia and growth cone development in neurons. Mol Biol Cell 16: 32-39, 2005 山下　俊秀　（千葉大学・大学院医学研究科） Hasegawa Y, Fujitani M, Hata K, Tohyama M, Yamagishi S, Yamashita T: Promotion of axon regeneration by MAG and Nogo through divergent signals downstream of Gi/G. J Neurosci 24: 6826-6832, 2004 Madura T, Yamashita T, Kubo T, Fujitani M, Hosokawa K, Tohyama M: Activation of Rho in the injured axons following spinal cord injury. EMBO Rep 5: 412-417, 2004 Aoki M, Yamashita T, Tohyama M: EphA receptors direct the differentiation of mammalian neural precursor cells through Ras/MAPK pathway. Chem J Biol 279: 32643-32650, 2004 Fujitani M, Yamagishi S, Che Y-H, Hata K, Kubo T, Ino H, Tohyama M, Yamashita T: P311 accelerates nerve regeneration of the axotomized facial nerve. J Neurochem 91: 737-744, 2004 Mizuno T, Yamashita T, Tohyama M: Chimaerins act downstream from neurotrophins in overcoming inhibition of neurite outgrowth by MAG. J Neurochem 91: 395-403, 2004 Tanaka H, Yamashita T, Yachi K, Fujiwara K, Yoshikawa K, Tohyama M: Cytoplasmic p21Cip1/WAF1 enhances axonal regeneration and functional recovery after spinal cord injury in rats. Neuroscience 127: 155-164, 2004 Tsuji L, Yamashita T, Kubo T, Madura T, Tanaka H, Hosokawa K, Tohyama M: A cell surface molecule containing LRR repeats and a FNIII domain, FLRT3, promotes neurite outgrowth. Biochem Biophys Res Commun 313: 1086-1091, 2004 Madura T, Yamashita T, Tsuji L, Hosokawa K, Tohyama M: Changes in mRNA of Slit-Robo GTPase activating protein 2 following facial nerve transection. Mol Brain Res 123: 76-80, 2004 Yamagishi S, Fujitani M, Hata K, Kitajo K, Mimura F, Abe H, Yamashita T: Wallerian degeneration involves Rho/Rho-kinase signaling. J Biol Chem 280: 20384-20388, 2005 Fujitani M, Kawai H,Proia RL, Kashiwagi A, Yasuda H, Yamashita T: Binding of soluble myelin-associated glycoprotein to specific gangliosides induces the association of p75NTR to lipid rafts and signal transduction. J Neurochem 94: 15-21, 2005 Fujitani M, Honda A, Hata K, Yamagishi S, Tohyama M, Yamashita T: Biological activity of neurotrophins is dependent on recruitment of Rac1 to lipid rafts. Biochem Biophys Res Commun 327: 150-154, 2005 Yamashita T, Fujitani M, Yamagishi S, Hata K, Mimura F: Multiple signals regulate axon regeneration through the Nogo receptor complex. Mol Neurobiol 32: 105-112, 2005 Yamashita T, Fujitani M, Hata K, Mimura F, Yamagishi S: Diverse functions of the p75 neurotrophin receptor. Anat Sci Int 80: 37-41, 2005 Doya H, Ohtori S, Takahashi K, Aoki Y, Ino H, Takahashi Y, Moriya H, Yamashita T: ERK MAP kinase activation in the dorsal root ganglion (DRG) and the spinal cord following DRG injury in rats. Spine 30: 2252-2256, 2005 山下　拓史　（広島大学・医学部・第３内科） Nakamura T, Yamashita H, Takahashi T, Nakamura S: Activated Fyn phosphorylates alpha-synuclein at tyrosine residue 125. Biochem Biophys Res Commun 280: 1085-1092, 2001 Nagano Y, Yamashita H, Nakamura T, Takahashi T, Kondo E, Nakamura S: Lack of binding observed between human alpha-synuclein and Bcl-2 protein family. Neurosci Lett 316: 103-107, 2001 Nakamura S, Takahashi T, Yamashita H, Kawakami H: Nicotinic acetylcholine receptors and neurodegenerative disease. Alcohol 24: 79-81, 2001 Nakamura T, Yamashita H, Nagano Y, Takahashi T, Avraham S, Avraham H, Matsumoto M, Nakamura S: Activation of Pyk2/RAFTK induces tyrosine phosphorylation of alpha-synuclein via Src-family kinases. FEBS Lett 521: 190-194, 2002 Takahashi T, Yamashita H, Nakamura T, Nagano Y, Nakamura S: Tyrosine 125 of alpha -synuclein plays a critical role for dimerization following nitrative stress. Brain Res 938: 73-80, 2002 山田　光則　（新潟大学・脳研究所） Yamada M, Tan C-F, Inenaga C, Tsuji S, Takahashi H: Sharing of polyglutamine localization by the neuronal nucleus and cytoplasm in CAG-repeat diseases. Neuropathol Appl Neurobiol 30: 665-675, 2004 Toyoshima Y, Yamada M, Onodera O, Shimohata M, Inenaga C, Fujita N, Morita M, Tsuji S, Takahashi H: SCA17 homozygote showing Huntington’s disease-like phenotype. Ann Neurol 55: 281-286, 2004 吉澤　利弘　（筑波大学・臨床医学系・神経内科） Yoshizawa T, Yoshida H, Shoji S: Differential susceptibility of cultured cell lines to aggregate formation and cell death produced by the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch. Brain Res Bull 56: 349-352, 2001 Ohye T, Ichinose H, Yoshizawa T, Kanazawa I, Nagatsu T: A new splicing variant for human tyrosine hydroxylase in the adrenal medulla. Neurosci Lett 312: 157-160, 2001 Hatakeyama S, Wakamori M, Ino M, Miyamoto N, Takahashi E, Yoshinaga T, Sawada K, Imoto K, Tanaka I, Yoshizawa T, Nishizawa Y, Mori Y, Niidome T, Shoji S: Differential nociceptive responses in mice lacking the alpha 1B subunit of N-type Ca2+ channels. NeuroReport 118: 2423-2427, 2001 Yoshizawa T, Kohno Y, Nissato S, Shoji S: Compound heterozygosity with 2 novel mutations in the HEXB gene produces adult Sandhoff disease presenting as a motor neuron disease phenotype. J Neurol Sci 195: 129-138, 2002 Nakayama J, Yoshizawa T, Yamamoto N, Arinami T: Mutation analysis of the calpastatin gene (CAST) in patients with Alzheimer's disease. Neurosci Lett 320: 77-80, 2002 Yoshida H, Yoshizawa T, Shibasaki F, Shoji S, Kanazawa I: Chemical chaperones reduce aggregate formation and cell death caused by the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch. Neurobiol Dis 10: 88-99, 2002 吉田　裕樹　（九州大学・生体防御医学研究所） Haraguchi M, Torii S, Matsuzawa S, Xie Z, Kitada S, Krajewski S, Yoshida H, Mak TW, Reed JC: Apoptotic protease activating factor 1 (Apaf-1)-independent cell death suppression by Bcl-2. J Exp Med 191: 1709-1720, 2000 Guo Z, Yikang S, Yoshida H, Mak TW, Zacksenhaus E: Inactivation of the retinoblastoma tumor suppressor induces apoptosis protease-activating factor-1 dependent and independent apoptotic pathways during embryogenesis. Cancer Res 61: 8395-8400, 2001 Hirao A, Kong YY, Matsuoka S, Wakeham A, Ruland J, Yoshida H, Liu D, Elledge SJ, Mak TW: DNA damage-Induced activation of p53 by the checkpoint kinase Chk2. Science 287: 1824-1827, 2000 Joza N, Susin SA, Daugas E, Stanford WL, Cho SK, Li CY, Sasaki T, Elia AJ, Cheng HY, Ravagnan L, Ferri KF, Zamzami N, Wakeham A, Hakem R, Yoshida H, Kong YY, Mak TW, Zuniga-Pflucker JC, Kroemer G, Penninger JM: Essential role of the mitochondrial apoptosis-inducing factor in programmed cell death. Nature 410: 549-554, 2001 Miyazaki K, Yoshida H, Sasaki M, Hara H, Kimura G, Mak TW, Nomoto K: Caspase-independent cell death and mitochondrial disruptions observed in the apaf1-deficient cells. J Biochem 129: 963-969, 2001 Hossain MS, Takimoto H, Ninomiya T, Yoshida H, Kishihara K, Matsuzaki G, Kimura G, Nomoto K: Characterization of CD4- CD8- CD3+ T-cell receptor-alphabeta+ T cells in murine cytomegalovirus infection. Immunology 101: 19-29, 2000 Ninomiya T, Takimoto H, Matsuzaki G, Hamano S, Yoshida H, Yoshikai Y, Kimura G, Nomoto K: Vgamma1+ gammadelta T cells play protective roles at an early phase of murine cytomegalovirus infection through production of interferon-gamma. Immunology 99: 187-194, 2000 Yada S, Nukina H, Kishihara K, Takamura N, Yoshida H, Inagaki-Ohara K, Nomoto K, Lin T: IL-7 prevents both caspase-dependent and -independent pathways that lead to the spontaneous apoptosis of i-IEL. Cell Immunol 208: 88-95, 2001 Yoshida H, Hamano S, Senaldi G, Covey T, Faggioni R, Mu S, Xia M, Wakeham AC, Nishina H, Potter J, Saris CJ, Mak TW: WSX-1 is required for the initiation of Th1 responses and resistance to L. major Infection. Immunity 15: 569-578, 2001 Ferry S, Matsuda M, Yoshida H, Hirata M: Inositol hexakisphosphate blocks tumor cell growth by activating apoptotic machinery as well as by inhibiting the Akt/NFkappaB-mediated cell survival pathway. Carcinogenesis 23: 2031-2041, 2002 Hara H, Takeda A, Takeuchi M, Wakeham AC, Itie A, Sasaki M, Mak TW, Yoshimura A, Nomoto K, Yoshida H: The apoptotic protease-activating factor 1-mediated pathway of apoptosis is dispensable for negative selection of thymocytes. J Immunol 168: 2288-2295, 2002 Haraguchi M, Tsujimoto H, Fukushima M, Higuchi I, Kuribayashi H, Utsumi H, Nakayama A, Hashizume Y, Hirato J, Yoshida H, Hara H, Hamano S, Kawaguchi H, Furukawa T, Miyazono K, Ishikawa F, Toyoshima H, Kaname T, Komatsu M, Chen ZS, Gotanda T, Tachiwada T, Sumizawa T, Miyadera K, Osame M, Noda T, Yamada Y, Akiyama S: Targeted deletion of both thymidine phosphorylase and uridine phosphorylase and consequent disorders in mice. Mol Cell Biol 22: 5212-5221, 2002 Inagaki-Ohara K, Takamura N, Yada S, Alnadjim Z, Liu E, Yu X, Yoshida H, Lin T: Radiation-induced crypt intestinal epithelial cell apoptosis in vivo involves both caspase-3-dependent and -independent pathways. Dig Dis Sci 47: 2823-2830, 2002 Kinjyo I, Hanada T, Inagaki-Ohara K, Mori H, Aki D, Ohishi M, Yoshida H, Kubo M, Yoshimura A: SOCS1/JAB is a negative regulator of LPS-induced macrophage activation. Immunity 17: 583-91, 2002 Sasaki M, Miyazaki K, Koga Y, Kimura G, Nomoto K, Yoshida H: Calcineurin-dependent mitochondrial disturbances in calcium-induced apoptosis of human immunodeficiency virus gp160-expressing CD4+ cells. J Virol 76: 416-420, 2002 Takayanagi H, Kim S, Koga T, Nishina H, Isshiki M, Yoshida H, Saiura A, Isobe M, Yokochi T, Inoue J, Wagner EF, Mak TW, Kodama T, Taniguchi T: Induction and activation of the transcription factor NFATc1 (NFAT2) integrate RANKL signaling in terminal differentiation of osteoclasts. Dev Cell 3: 889-901, 2002 Yoshida H, Okada Y, Kinoshita N, Hara H, Sasaki M, Sawa H, Nagashima K, Mak TW, Ikeda K, Motoyama N: Differential requirement for Apaf1 and Bcl-X(L) in the regulation of programmed cell death during development. Cell Death Differ 9: 1273-1276, 2002 若松　延昭　（愛知県心身障害者コロニー発達障害研究所） Wakamatsu N, Yamada Y, Yamada K, Ono T, Nomura N, Taniguchi H, Kitoh H, Mutoh N, Yamanaka T, Mushiake K, Kato K, Sonta S, Nagaya M: Mutations in SIP1, encoding Smad interacting protein-1, cause a form of Hirschsprung disease. Nat Genet 27: 369-370, 2001 Yamada Y, Miura K, Kumagai T, Hayakawa C, Miyazaki S, Matsumoto A, Kurosawa K, Nomura N, Taniguchi H, Sonta S, Yamanaka T, Wakamatsu N: Molecular analysis of Japanese patients with Rett syndrome: Identification of five novel mutations and genotype-phenotype correlation. Hum Mutat 18: 253, 2001 Yamada K, Yamada Y, Nomura N, Miura K, Wakako R, Hayakawa C, Matsumoto A, Kumagai T, Yoshimura I, Miyazaki S, Kato K, Sonta S, Ono H, Yamanaka T, Nagaya M, Wakamatsu N: Nonsense and frameshift mutations in ZFHX1B, encoding Smad-Interacting protein 1, cause a complex developmental disorder with a great variety of clinical features. Am J Hum Genet 69: 1178-1185, 2001 Nagaya M, Kato J, Niimi, N, Tanaka S, Wakamatsu N: Clinical feature of a form of Hirschsprung’s disease caused by a novel genetic abnormality. J Pediar Surg 37: 1117-1122, 2002 Yoneda M, Fujita T, Yamada Y, Yamada K, Fujii A, Inagaki T, Nakagawa H, Kishikawa M, Shimada A, Nagaya M, Azuma T, Kuriyama M, Wakamatsu N: Late infantile Hirschsprung disease-mental retardation syndrome with a 3-bp deletion in ZFHX1B. Neurology 59: 1637-1640, 2002

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