辻　省次　（新潟大学・脳研究所）

1.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

2.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

3.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

4.Zhao C, Takita J, Tanaka Y, Setou M, Nakagawa T, Takeda S, Yang HW, Terada S, Nakata T, Takei Y, Saito M, Tsuji S, Hayashi Y, Hirokawa N: Charcot-Marie-Tooth disease type 2A caused by mutation in a microtubule motor KIF1Bβ. Cell 105: 587-597, 2001

5.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, Saito K, 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

6.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

7.Takahashi H, Egawa H, Piao Y-S, Hayashi S, Yamada M, Shimohata T, Oyanagi K, Tsuji S: Neuronal nuclear alterations in dentatorubral-pallidoluysian atrophy: ultrastructural and morphometric studies of the cerebellar granule cells. Brain Res 919:12-19, 2001

8.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　in press

池田穰衛　（東海大学・総合医学研究所）

1.Wang F, Corbett D, Osuga H, Osuga S, Ikeda JE, 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 

2.Uchida M, Shimatsu Y, Onoe K, Matsuyama N, Niki R, Ikeda JE, Imai H: Production of transgenic miniature pigs by pronuclear microinjection. Transgenic Res 10: 577-582, 2001

3.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 BA, Sagie T, Skaug J, Nasir J, Brown RH Jr, Scherer SW, Rouleau GA, Hayden MR, Ikeda JE: A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nat Genet 29: 166-173, 2001

4.Hadano S, Yanagisawa Y, Skaug J, Fichter K, Nasir J, Martindale D, Koop BF, Scherer SW, Nicholson DW, Rouleau GA, Ikeda JE, 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

岩坪　威　（東京大学・大学院薬学系研究科）

1.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

2.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

3.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

4.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

5.Piao Y-S, Hayashi S, Hasegawa M, Wakabayashi K, Yamada M, Yoshimoto M, Ishikawa A, Iwatsubo T, Takahashi T: Co-localization of α-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

6.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

7.Mann DMA, McDonagh AM, Pickering-Brown SM, Kowa H, Iwatsubo T: Amyloid b protein deposition in patients with frontotemporal lobar degeneration: relationship to age and apolipoprotein E genotype. Neurosci Lett 304: 161-164, 2001

8.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

9.Mann DMA, Pickering-Brown SM, Takeuchi A, Iwatsubo T: Amyloid angiopathy and variability in amyloid b deposition is determined by mutation position in presenilin-1 linked Alzheimer’s disease. Am J Pathol 158: 2165-2175, 2001

10.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

11.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

12.Lambert J-C, Mann D, Harris J M, 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

13.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

14.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

15.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

16.Kahle PJ, Neumann M, Ozmen L, Muller V, Odoy S, Jacobsen H, Iwatsubo T, Trojanowski JQ, Takahashi H, Wakabayashi K, Bogdanovic N, Riederer P, Kretzschmar HA, 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

17.Ikegaya Y, 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

18.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

19.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 in press

20.Hayashi S, Toyoshima Y, Hasegawa M, Umeda Y, Wakabayashi K, Tokiguchi S, Iwatsubo T, Takahashi H: Late-onset frontotemporal dementia with glial-predominant tau pathology and a novel exon 1 (Arg5His) tau gene mutation. Ann Neurol in press

21.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 in press

祖父江元　（名古屋大学・大学院医学研究科）

1.Kobayashi Y, Sobue G: Protective effect of chaperones on polyglutamine diseases. Brain Res Bull 56: 165-168, 2001

2.Adachi H, Kume A, Li M, Nakagomi Y, Niwa H, Do J, Sang C, Kobayashi Y, Doyu M, Sobue G: Transgenic mice with an expanded 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

3.Hishikawa N, Hashizume Y, Yoshida M, Sobue G: Widespread occurrence of argyrophilic glial inclusions in Parkinson's disease. Neuropathol Appl Neurobiol 27: 362-372, 2001

4.Qiao S, Iwashita T, Furukawa T, Yamamoto M, Sobue G, Takahashi M: Differential effects of leukocyte common antigen-related protein on biochemical and biological activities of RET-MEN2A and RET-MEN2B mutant proteins. J Biol Chem 276: 9460-9467, 2001

5.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 Sjogren's syndrome. J Neurol Neurosurg Psychiat 71: 488-492, 2001

6.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

7.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

8.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

9.Watanabe H, Ieda T, Katayama T, Takeda A, Aiba I, Doyu M, Hirayama M, Sobue G: Cardiac (123)I-meta-iodobenzylguanidine (MIBG) uptake in dementia with Lewy bodies: comparison with Alzheimer's disease. J Neurol Neurosurg Psychiat 70: 781-783, 2001

10.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

11.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 vasculitic neuropathy. Muscle Nerve 24: 830-833, 2001

12.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: 706-13, 2001

13.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

14.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 [N epsilon-(carboxymethyl)lysine]] in Alzheimer’s disease brains. Acta Neuropathol 101: 27-35, 2001

15.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

16.Ikeda S, Nakazato M, Ando Y, Sobue G: Familial transthyretin type amyloid polyneuropathy in Japan: clinical and genetic heterogeneity. Neurology in press

17.Mori K, Hattori N, Sugiura M, Koike H, Misu K, Ichimura M, Hirayama M, Sobue G: Chronic inflammatory demyelinating polyneuropathy presenting with features of GBS.　 Neurology in press

18.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 in press

19.Yoshihara T, Ishigaki S, Yamamoto M, Liang Y, Niwa J, Takeuchi H, Doyu M, Sobue G: Differential expression of inflamation- and apoptosis-related genes in spinal cord of a mutant SOD 1 transgenic mouse model of familial amyotrophic lateral sclerosis. J Neurochem in press

20.Watanabe H, Saito Y, Terao S, Ando T, Kachi T, Mukai E, Aiba I, Abe Y, Tamakoshi A, Doyu M, Hirayama M, Sobue G: Progression and prognosis in multiple system atrophy: An analysis of 230 Japanese patients. Brain in press

21.Hideyuki Takeuchi, Yasushi Kobayashi, Tsuyoshi Yoshihara, Jun-ichi Niwa, Manabu Doyu, Kenzo Ohtsuka, Gen Sobue: Hsp70 and Hsp40 improve neurite outgrowth and suppress intracytoplasmic aggregate formation in cultured neuronal cells expressing mutant SOD1. Brain Res in press

22.Iwai K, Yamamoto M, Yoshihara T, Sobue G: Anticipation in familial amyotrophic lateral sclerosis with SOD1-G93S mutation. J Neurol Neurosurg Psychiat in press

23.Dei R, Takeda A, Niwa H, Li M, Nakagaomi Y, Watanabe M, Inagaki T, Washimi Y, Yasuda Y, Horie K, Miyata T, Sobue G: Lipid peroxidation and advanced glycation endproducts in the brain in normal aging and in Alzheimer’s disease. Acta Neuropathol in press

木村洋子　（東京都臨床医学総合研究所）

1.Kimura Y, Koitabashi S, Kakizuka A, Fujita T: Initial process of polyglutamine aggregate formation in vivo. Genes Cells 6: 887-897, 2001

2.Hirabayashi M, Inoue K, Tanaka K, Nakadate K, Ohsawa Y, Kamei Y, Popiel AH, Sinohara A, Iwamatsu A, Kimura Y, Uchiyama Y, Hori S, Kakizuka A: VCP/p97 in abnormal protein aggregates, cytoplasmic vacuoles, and cell death, phenotypes relevant to neurodegeneration.　 Cell Death Differ 8: 977-984, 2001

水野美邦　（順天堂大学・医学部）

1.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

2.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

3.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 Neruochem 78: 42-54, 2001

4.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

5.Miwa H, Mizuno Y: Capgras syndrome in Parkinson’s disease. J Neurol 248: 804-805, 2001

6.Miwa H, Miwa T, Imai H, Mizuno Y: Obsessive compulsive disorder-like behavioral changes in pure akinesia. Parkinsonism Relat Disord 7: 315-317, 2001

7.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

8.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

9.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

10.Okuma Y, Tanaka R, Fujishima K, Kobayashi T, Mizuno Y: Cortical reflex action myoclonus in neurosyphilis. Eur Neurol 45: 193-194, 2001

11.Okuma Y, Mizuno Y: Myoclonic tremor in patients with parkinsonian-type multiple system atrophy. Mov Disord 16: 378-379, 2001

12.Okuma Y, Fujishima K, Machida Y, Inagaki N, Sugita Y, Mizuno Y: Proprispinal negative myoclonus. Eur Neurol 46: 99-101, 2001

13.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

14.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

15.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

16.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 rato brain. J Neurochem 77: 1561-1568, 2001

17.Miwa H, Mizuno Y: Enlargements of somatosensory-evoked potentials in progressive supranuclear palsy. Acta Neurol Scand in press

18.Miwa H, Mizuno Y, Kondo T: Familial hemifacial spasm. Report of cases and review of literature. J Neurol Sci in press

青木正志　（東北大学・医学部）

1.Aoki M, Liu J, Richard I, Keers SM, Marchand S, Bourg N, McKenna-Yasek D, Arahata K, Bushby K, Beckmann J, Brown RH Jr: Genomic organization and novel mutations in dysferlin gene in Miyoshi myopathy and limb girdle dystrophy type 2B. Neurology 57: 271-278, 2001

2.O’Neill GN, Aoki M, Brown RH Jr: ABCD1 translation-initiator mutation demonstrates genotype-phenotype correlation for AMN. Neurology 57: 1956-1962, 2001

3.Nagai M, Aoki M, Miyoshi I, Kato M, Pasinelli P, Kasai N, Brown RH Jr, Itoyama Y: Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: associated mutations develop motor neuron disease. J Neurosci 21: 9246-9254, 2001

4.Kato M, Aoki M, Ohta, M, Nagai M, Ishizaki F, Nakamura S, Itoyama Y: Marked reduction 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

5.Matsuda C, Hayashi YK, Ogawa M, Aoki M, Murayama K, Nishino I, Nonaka I, Arahata K, Brown RH Jr: The sarcolemmal proteins dysferlin and caveolin-3 interact in skeletal muscle. Hum Mol Genet 10: 1761-1766, 2001

6.Trotti D, Aoki M, Pasinelli P, Berger UV, Danbolt NC, Brown RH Jr, Hediger MA: Amyotrophic lateral sclerosis-linked glutamate transporter mutant has impaired glutamate clearance capacity. J Biol Chem 276: 576-582, 2001

7.Tsuchiya K, Matsunaga T, Aoki M, Haga C, Ooe K, Abe K, Ikeda K, Nakano I: Familial amyotrophic lateral sclerosis with posterior column degeneration and basophilic inclusion bodies: a clinical, genetic and pathological study. Clin Neuropathol 20: 53-59, 2001

8.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

石川欽也　（東京医科歯科大学・医学部 ）

1.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

2.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.

3.Sodeyama N, Iwata T, Ishikawa K, Mizusawa H, Yamada M, Itoh Y, Otomo E, Matsushita M, Komatsuzaki Y: Very early onset Alzheimer’s disease with spastic paraparesis associated with a novel presenilin 1 mutation (Phe237Ile). J Neurol Neurosurg Psychiat 71: 556-557, 2001

4.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 in press

石黒啓司　（藤田保健衛生大学・総合医科学研究所）

1.Ishiguro H, Yamada K, Sawada H, Nishii K, Ichino N, Sawada M, Kurosawa Y, Matsushita N, Kobayashi K, Goto J, Hishida 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

2.Yoshinaka T, Nishii K, Yamada K, Sawada H, Nishiwaki E, Yoshino K, Ishiguro H, Higashiyama S: Identification and characterization of novel mouse and human ADAM33s with potential metalloprotease activity. Gene 282: 227-236, 2002

3.Asakura M, Kitakaze M, Takashima S, Liao Y, Ishikura F, Yoshinaka T, Ohmoto H, Node K, Yoshino K, Ishiguro H, Asanuma H, Sanada S, Matsumura Y, Takeda H, Beppu S, Tada M, Hori M, Higashiyama S: Cardiac hypertrophy is inhibited by antagonism of ADAM12 processing of HB-EGF: Metalloprotease inhibitors as a potential new therapy for cardiac hypertrophy. Nat Med 8: 35-40, 2002

4.Ichino N, Yamada K, Nishii K, Sawada H, Nagatsu T, Ishiguro H: Increase of transcriptional levels of egr-1 and nur77 genes due to both nicotine treatment and withdrawal in pheochromocytoma cells. J Neural Transm in press

一瀬　宏　（藤田保健衛生大学・総合医科学研究所）

1.Zabetian CP, Anderson GM, Buxbaum SG, Elston RC, Ichinose H, Nagatsu T, KIm K-S, Kin C-H, 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

2.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 Esherichia coli. Heterocycles 54: 283-290, 2001

3.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

4.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

5.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

6.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

7.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

8.Kawasaki H, Suemori H, Mizuseki K, Watanabe K, Urano F, Ichinose H, Haruta M, Takahashi M, Yoshikawa K, Nishikawa S-i, 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

大野耕策　（鳥取大学・医学部）

1.Tsukamoto H, Yamamoto T, Nishigaki T, Sakai N, Nanba E, Ninomiya H, Ohno K, Inui K, Okada S: SSCP analysis by RT-PCR for the prenatal diagnosis of Niemann-Pick disease type C. Prenat Diagn 21: 55-57, 2001

2.Sawamura N, Gong J-S, Garver WS, Ninomiya H, Ohno K, Yanagisawa K, Michikawa M: Site-specific phosphorylation of tau accompamed by activation of mitogen-activated protein kinase (MAPK) in brains of Niemann-Pick type C model mice. J Biol Chem 276: 10314-10319, 2001

3.Higaki K, Ninomiya H, Sugimoto Y, Suzuki T, 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

4.Tominaga L, Ogawa Y, Taniguchi M, Ohno K, Matsuda J, Oshima A, Suzuki Y, Nanba E: Galactonojirimycin derivatives restore mutant human β-galactosidase activities expressed in fibroblasts from enzyme deficient knockout mouse. Brain Dev 23: 284-287, 2001

5.Yamada A, Saji M, Ukita Y, Taniguchi M, Ninomiya H, Ohno K: Progressive neuronal loss in the ventral posterior lateral and medial nuclei of the thalamus in Niemann-Pick disease type V mouse brain. Brain Dev 23: 288-297, 2001

6.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

7.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

8.Watabe K, Ida H, Uehara K, Oyanagi K, Sakamoto T, Tanaka J, Garaver WS, Miyawaki S, Ohno K, Eto Y: Establishment and characterization of immortalized Schwann cells from murine model of Niemann-Pick disease type C. J Periph Nerv Syst 6: 85-94, 2001

9.Sugimoto Y, Ninomiya H, Ohsaki Y, Higaki K, Danies JP, Ioannou YA, Ohno K: Accumulation of cholera toxin and GM1 ganglioside in the early endosome of Niemann-Pick C1 deficient cells. Prac Natl Acad Sci USA 98: 12391-12396, 2001

郭　伸　（東京大学・医学部）

1.Yoshimura M, Yamamoto T, Sone-Isoo N, Imafuku I, Momose T, Shirouzu I, Kwak S, Kanazawa I: Hemiparkinsonism in a patient with a mesencephalic tumor. J Neurol Sci in press

木山博資　（大阪市立大学・大学院医学研究科）

1.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 in press

2.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

3.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 Chem 276: 36632-36638, 2001

4.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

5.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

6.Takeda M, Mori F, Yoshida A, Takamiya A, Nakagomi S, Sato E, Kiyama H: Constitutive nitric oxide synthase is associated with retinal vascular permeability in early diabetic rats. Diabetrogia 44: 1043-1050, 2001

7.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

8.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

9.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

10.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

11.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

12.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

小池達郎　（北海道大学・大学院理学研究科）

1.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

2.Saitoh T, Tanaka S, Koike T: Rapid induction and Ca²⁺ influx-mediated suppression of vitamin D3 up-regulated protein 1 (VDUP1) mRNA in cerebellar granule neurons undergoing apoptosis. J Neurochem 78: 1267-1276, 2001

3.Origasa M, Tanaka S, Suzuki K, Tone S, Lim B, Koike T: Activation of a novel microglial gene encoding a lysosomal membrane protein in response to neuronal apoptosis. Mol Brain Res 88: 1-13, 2001

4.Tanaka S, Koike T: Activation of protein kinase C delays apoptosis of nerve growth factor-deprived rat sympathetic neurons through a Ca²⁺-influx dependent mechanism. Neurosci Lett 313: 9-12, 2001

5.Kaya N, Tanaka S, Koike T: ATP selectively suppresses the synthesis of the inflammatory protein, MRF-1, through Ca²⁺-influx via P2X7-receptors in cultured microglia. Brain Res in press

小阪　淳　（岡山大学・大学院医歯学総合研究科）

1.Wakabayashi T, Kosaka J, Honmura S: Up-regulation of Hrk, a regulator of cell death, in retinal ganglion cells of axotomized rat retina. Neurosci Lett 318: 77-88, 2002

坂口末廣　（長崎大学・大学院医学研究科）

1.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

2.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

佐野　輝　（愛媛大学・医学部）

1.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 Genet 28: 121-122, 2001

2.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

下濱　俊　（京都大学・大学院医学研究科）

1.Kakimura J, Kitamura Y, Taniguchi T, Shimohama S, Gebicke-Haerter PJ: Bip/GRP78-induced production of cytokines and uptake of amyloid-β(1-42) peptide in microglia. Biochem Biophys Res Commun 281: 6-10, 2001

2.Urushitani M, Nakamizo T, Inoue R, Sawada H, Kihara T, Honda K, Akaike A, Shimohama S: N-methyl-D-apartate receptor-mediated mitochondrial Ca²⁺ overload in acute excitotoxic motor neurons death: A mechanism distinct from chronic neurotoxicity after Ca²⁺ influx. J Neurosci Res 63: 377-387, 2001

3.Shimohama S, Kihara T: Nicotinic receptor-mediated protection against beta amyloid neurotoxicity. Biol Psychiat 49: 233-239, 2001

4.Kitamura Y, Sanada H, Kakimura J, Ishida Y, Shimohama S, Taniguchi T: Decrease of tyrosine hydroxylase-, α-synuclein- and parkin-expressing neurons in the substantia nigra of MPTP-treated C57BL/6N mice. Biogenic Amines 16: 127-136, 2001

5.Kitamura Y, Ishida Y, Takata K, Kakimura J, Mizutani H, Shimohama S, Akaike A, Taniguchi T: α-Synuclein proteins is not scavenged in neuronal loss induced by kainic acid or focal ischemia. Brain Res 898: 181-185, 2001

6.Kageyama T, Kawamata J, Shimohama S: Coexistence of two different type I repeats. Arch Neurol 58: 1022, 2001

7.Ibi M, Sawada H, Nakanishi M, Kume T, Katsuki H, Kaneko S, Shimohama S, Akaike A: Protective effects of 1 α, 25-(OH)₂D₃ against the neurotoxicity of glutamate and reactive oxygen species in mesencephalic culture. Neuropharmacology 40: 761-771, 2001

8.Kihara T, Shimohama S, Sawada H, Honda K, Nakamizo T, Shibasaki H, Kume T, Akaike A: Alpha7 nicotinic receptor transduces signals to PI3 kinase to block Aβ-induced neurotoxicity.　 J Biol Chem 276: 13541-13546, 2001

9.Honda K, Shimohama S, Sawada H, Kihara T, Nakamizo T, Shibasaki H, Akaike A: Nongenomic antiapoptotic signal transduction by estrogen in cultured cortical neurons. J Neurosci Res 64: 466-475, 2001

10.Hirai K, Aliev G, Nunomura A, Fujioka H, Russell RL, Atwood CS, Johnson AB, KressY, Vinters HV, Tabaton M, Shimohama S, Cash AD, Siedlak SL, Harris PLR, Jones PK, Petersen RB, Perry G, Smith MA: Mitochondrial abnormalities in Alzheimer disease. J Neurosci 21: 3017-3023, 2001

11.Nunomura A, Perry G, Aliev G, Hirai K, Takeda A, Balraj EK, Jones PK, Ghanbari H, Wataya T, Shimohama S, Chiba S, Atwood CS, Petersen RB, Smith MA: Oxidative damage is the earliest event in Alzheimer disease. J Neuropathol Exp Neurol 60: 759-767, 2001

12.Oeda T, Shimohama S, Kitagawa N, Kohno R, Imura T, Shibasaki H, Ishii N: Oxidative stress causes abnormal accumulation of familial amyotrophic lateral sclerosis-related mutant SOD1 in transgenic C. elegans. Hum Mol Genet 10: 2013-2023, 2001

13.Zhu X, Rottkamp CA, Hartzler A, Sun Z, Takeda A, Boux H, Shimohama S, Perry G, Smith MA: Activation of MKK6, an upstream activator of p38, in Alzheimer’s disease. J Neurochem 79: 311-318, 2001

14.Tanino H, Kusuda T, Kishimoto A, Shimohama S, Fujimoto S: Alterations of phospholipase C isozymes in rat cerebral cortex through hyperoxia. Biol Pharm Bull 24: 1241-1245, 2001

15.Shimohama S, Tanino H, Fujimoto S: Differential subcellular localization of caspase family proteins in the adult rat brain. Neurosci Lett 315: 125-128, 2001

16.Shimohama S, Tanino H, Fujimoto S: Differential expression of rat brain caspase family proteins during development and aging. Biochem Biophys Res Commun 289: 1063- 1066, 2001

17.Urushitani M, Shimohama S: The role of nitric oxide in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2: 71-81, 2001

18.Tsuji T, Shimohama S: Analysis of the proteomic profiling of brain tissue in Alzheimer’s disease. Dis Markers 17: 247-257, 2001

19.Castellani RJ, Perry G, Siedlak SL, Nunomura A, Shimohama S, Zhang J, Montine T, Sayre LM, Smith MA: Hydroxynonenal adducts indicate a role for lipid peroxidation in neocortical and brainstem Lewy bodies. Neurosci Lett 319: 25-28, 2002

20.Wataya T, Nunomura A, Smith MA, Siedlak SL, Harris PLR, Shimohama S, Szweda LI, Kaminski MA, Price DL, Cleveland DW, Sayre LM, Perry G: High molecular weight neurofilament proteins are physiological substrates of adduction by the lipid peroxidation product hydroxynonenal. J Biol Chem 277: 4644-4648, 2002

21.Yamamoto K, Hashimoto K, Nakano M, Shimohama S, Kato N: A distinct form of calcium release downregulates membrane excitability in neocortical pyramidal cells. Neuroscience in press

22.Kawamata J, Shimohama S: Association of novel and established polymorphisms in neuronal nicotinic acetylcholine receptors with sporadic Alzheimer’s disease. J Alz Dis in press

23.Yamamoto K, Nakano M, Hashimoto K, Shimohama S, Kato N: Emergence of a functional coupling between insotol-1,4,5-trisphosphate receptors and calcium channels in developing neocortical pyramidal neurons. Neuroscience in press

24.Kakimura J-I, Kitamura Y, Umeki M, Suzuki S, Shibagaki K, Taniguchi T, Nomura Y, Gebicke-Haerter PJ, Smith MA, Perry G, Shimohama S: Microglial activation and amyloid-β clearance induced by exogenous heat-shock proteins. FASEB J in press

25.Kume T, Asai N, Nishikawa H, Mano N, Terauchi T, Taguchi R, Shirakawa H, Osakada F, Mori H, Asakawa N, Yonaga M, Nishizawa Y, Sugimoto H, Shimohama S, Katsuki H, Kaneko S, Akaike A: Isolation of a novel diterpenoid substance with potent neuroprotective activity from fetal calf serum. Proc Nat Acad Sci USA in press

鈴木俊顕　（東京都臨床医学総合研究所）

1.Matsuda N, Suzuki T, Uchimiya H, Tanaka K, Nakano A: Rma1, a novel type of RING finger protein conserved from Arabidopsis to human, is a membrane-bound ubiquitin ligase. J Cell Sci 114: 1949-1957, 2001

2.Niwa J, Ishigaki S, Doyu M, Kato K, Suzuki T, Tanaka K, Sobue G: A novel human RING-finger/IBR family protein, Dorfin, resides in centrosome and mediates ubiquitin ligase activity. Biochem Biophys Res Commun 281: 706-713, 2001

3.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: 4003-4012, 2001

4.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

5.Takabe W, Kodama T, Hamakubo T, Tanaka K, Suzuki T, Aburatani H, Noguchi N: Regulation of the expression and function of proteasome α-type subunits by the anti-atherogenic compounds in human endothelial cells. J Biol Chem 276: 40497-40501, 2001

6.Kadoya K, Yamamoto H, Suzuki T, Yukita A, Fukui A, Michine T, Asahara T, Tananak K, Asashima NM, Kikuchi A: Desumolyation activity of Axam, a novel Axin-binding protein, is involved in downregulation of β-catenin. Mol Cell Biol in press

高橋良輔　（理化学研究所・脳科学総合研究センター）

1.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

2.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

3.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

4.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

5.Suzuki Y, Imai Y, Nakayama H, Takahashi K, Takio K, Takahashi R: A serine protease, HtrA2, is released from the mitochondria and interacts with XIAP, inducing cell death. Mol Cell 8: 613-21, 2001

6.Araya R, Takahashi R, Nomura Y: Yeast two-hybrid screening using consttitutive-active caspase-7 as bait in the identification of PA28γ as an effector caspase substrate. Cell Death Differ in press

程久美子　（東京大学・理学部）

1.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, reaper. Mol Cell Biol Res Commun 4: 307-312, 2001

2.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

3.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

中村浩一郎　（東京大学・大学院医学系研究科）

1.Nakamura K, Jeong SY, 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

中村重信　（広島大学・医学部）

1.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

2.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

3.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

4.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

5.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

6.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

7.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

8.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

9.Kato M, Aoki M, Ohta M, Nagai M, Ishizaki F, Nakamura S, Itoyama Y: Marked 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

10.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

11.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

12.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

13.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. Neuropath Appl Neurobiol 27:180-188, 2001

14.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　　

15.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

16.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

17.Takahashi T, Yamashita H, Nagano Y, Nakamura T, Nakamura S: Tyrosine 125 of α-synuclein plays a critical role for dimerization following nitrative stress. Brain Res in press

18.Inoue K, Mimori Y, Nakamura S: N10 potential as an antedromic motor evoked potential in a median nerve short-latency somatosensory evoked potential study. J Clin Neurophysiol in press

19.Shimizu ME, Ishizaki F, Nakamura S: Results of a home exercise program for patients with osteoporosis resulting from neurological disorders. Hiroshima J Med Sci in press

20.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 in press

貫名信行　（理化学研究所・脳科学総合研究センター ）

1.Iwata A, Miura S, Kanazawa I, Sawada M, Nukina N: α-Synuclein forms a complex with transcription factor Elk-1. J Neurochem 77: 239-252, 2001

2.Jana N-R, Zemskov E-A, Wang G-H, 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

3.Iwata A, Maruyama M, Kanazawa I, Nukina N: α-Synuclein affects the MAP kinase pathway and accelerates cell death. J Biol Chem 276: 45320-45329, 2001

4.Tanaka M, Morishima I, Akagi T, Hashikawa T, Nukina N: Intra- and intermolecular β-pleated sheet formation in glutamine-repeat inserted myoglobin as a model for polygulutamine diseases. J Biol Chem 276: 45470-45475, 2001

長谷川一子　（北里大学・医学部）

1.Hasegawa K, Sakai F, Kowa H: The phenomenon of nocturnal dystonia in Parkinoson's disease. Eur Neurol 8(suppl 1): 1-5, 2001

2.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

3.Hasegawa K, Funayama M, Matsuura N, Furusawa H, Sakai F, Kowa H, Obata F: Analysis of alpha-synuclein, parkin, tau, and UCH-L1 in a Japanese family with autosomal dominant parkinsonism. Eur Neurol 46: 20-24, 2001

4.Urakami K, Wada K, Arai H, Sasaki H, Kanai M, Shoji M, Ishizu H, Kashihara K, Yamamoto M, Tsuchiya-Ikemoto K, Morimatsu M, Takashima H, 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

5.Yokoyama T, Kusunoki JI, Hasegawa K, Sakai H, Yagishita S: Distribution and dynamic process of neuronal cytoplasmic inclusion (NCI) in MSA: A correlation of the density of NCI and the degree of involvement of the pontine nuclei. Neuropathology 21: 145-154, 2001

6.Tsuchiya K, Ikeda M, Hasegawa K, Fukui T, Kuroiwa T, Haga C, Oyanagi S, Nakano I, Matsushita M, Yagishita S, Ikeda K: Distribution of cerebral cortical lesions in Pick's disease with Pick bodies: a cliniclpathological study of six autopsy cases showing unusual clinical presentations. Acta Neuropathol 102: 553-571, 2001

7.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 in press

久永真市　（東京都立大学・理学研究科）

1.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

2.Chang W, Gruber D, Chari S, Kitazawa H, Hamazumi Y, Hisanaga S, Bulinski J C: Phosphorylation of MAP4 affects microtubule properties and cell cycle progression. J Cell Sci 114: 2879-2887, 2001

3.Bibb JA, Nishi A, O’Callaghan JP, Ule J, Lan M, Snyder GL, Horiuchi A, Saito T, Hisanaga S, Czernik AJ, Nairn AC, Greengard P: Phosphorylation of protein phosphatase inhibitor-1 by Cdk5. J Biol Chem 276: 14490-14497, 2001

4.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

5.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

船越　洋　（大阪大学・大学院医学系研究科）

1.Funakoshi H, Nakamura T: Identification of HGF-like protein as a novel neurotrophic factor for avian dorsal root ganglion sensory neurons. Biochem Biophys Res Commun 283: 606-612, 2001

2.Nakamura K, Funakoshi H, Tokunaga F, Nakamura T: Molecular cloning of a mouse scavenger receptor with C-type lectin (SRCL)(1), a novel member of the scavenger receptor family. Biochim Biophys Acta 1522: 53-58, 2001

3.Nakamura K, Funakoshi H, Miyamoto K, Tokunaga F, Nakamura T: Molecular cloning and functional characterization of a human scavenger receptor with C-type lectin (SRCL), a novel member of a scavenger receptor family. Biochem Biophys Res Commun 280: 1028-1035, 2001

4.Kishi YA, Funakoshi H, Matsumoto K, Nakamura T: Molecular cloning, expression and partial characterization of Xksy, a Xenopus member of the Sky family of receptor tyrosine kinases. Gene in press

5.Funakoshi H, Yonemasu T, Nakano T, Matumoto K, Nakamura T: Identification of Gas6, a putative ligand for Sky and Axl receptor tyrosine kinases, as a novel neurotrophic factor for hippocampal neurons. J Neurosci Res in press

堀江秀典　（早稲田大学・先端バイオ研究所）

1.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

2.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

3.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

4.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 in press

5.Saito H, Sango K, Horie H aori Takeshita K, Ikeda H, Ishigatsubo Y, Ishikawa Y: Trachea enhances neurite regeneration from adult rat nodose ganglia in vitro. Life Sci in press

6.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 in press

松井秀樹　（岡山大学・大学院医歯学総合研究科）

1.Matsushita M, Tomizawa K, Moriwaki M, Li S-T, Terada H, Ohmoto T, Matsui H: A high efficiency protein transduction system proving the role of PKA in long lasting LTP. J Neurosci 21: 6000-6007, 2001

2.Kurazono S, Okamoto M, Mori S, Matsui H: Recombinant core protein fragment of phosphacan, a brain specific chondroitin sulfate proteoglycan, promote excitotoxic cell death of cultured rat hippocampal neurons. Neurosci Lett 304: 169-172, 2001

3.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

4.Tomizawa K, Ohta J, Matsushita M, Moriwaki A, Li S-T, Takei K, Matsui H: Cdk5/P³⁵ regulates neurotransmitter release through phosphorylation and downregulation of P/Q-type voltage-dependent calcium channel activity. J Neurosci in press

松山知弘　（兵庫医科大学・医学部）

1.Hirata A, Yoshida S, Inoue N, Matsumoto K, Ninomiya A, Taniguchi M, Matsuyama T, Kato K, Iizaka 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

2.Tamatani M, Matsuyama T, Yamaguchi A, Mitsuda N, Tsukamoto Y, Taniguchi M, Ho Che Y, 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

3.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

4.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

丸山和佳子　（国立療養所中部病院・長寿医療研究センター）

1.Uezono T, Matsubara K, Maruyama W, Naoi M, Shimizu K, Saito O, Ogawa K, Mizukami H, N. 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

2.Toth B E, Homicsko K, Radnai B,Maruyama W, DeMaria J E, Vecsernyes M, Fekete M I K, Fülöp F, Naoi M, Freeman M E, Nagy M:　 Salsolinol is a putative endogenous neuro-intermediate lobe prolactin releasing factor. J Neuroendocrinol 13: 1-14, 2001

3.Naoi M, Maruyama W: Future neuroprotection in Parkinson’s disease. Parkinsonism Relat Disord 8: 139-145, 2001

4.Maruyama W, Akao Y, Youdim M B H, Davis B A, 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

5.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

6.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

7.Oh-hashi K, Maruyama W, Isobe K: Peroxynitrite induces gadd34, 45 and 153 via p38 MAPK in human neuroblastoma SH-SY5Y cells. Free Radc Biol Med 30: 213-221, 2001

8.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

9.Maruyama W, Akao Y, Carrillo M C, Kitani K, Youdim M B H, Naoi M: Neuroprotection by propargylamines in Parkinson’s disease: Suppression of apoptosis and induction of pro-survival genes. Neurotoxcol Teratol in press

10.Maruyama W, Takahashi T, Youdim M, Naoi M: The anti-parkinson drug, rasagiline, prevents apoptotic DNA damage induced by peroxynitrite in human dopaminergic neuroblastoma SH-SY5Y cells. J Neural Transm in press

11.Maruyama W, Oya-Ito T, Shamoto-Nagai M, Osawa T, Naoi M: Glyceraldehyde-3-phospate dehydrogenase is translocated into nuclei through Golgi apparatus during apoptosis induced by 6-hydroxydopamine in human dopaminergic SH-SY5Y cells. Neurosci Lett in press

12.Yamamoto T, Maruyama W, Kato Y, Yi H, Shamoto-Nagai M, Tanaka M, Sato Y, Naoi M: Selective nitration of mitochondrial complex I by peroxynitrite: Involvement in mitochondria dysfunction and cell death of dopaminergic SH-SY5Y cells. J Neural Transm in press

三浦正幸　（理化学研究所・脳科学総合研究センター）

1.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 in press

2.Mochizuki H, Hayakawa H, Migita-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

3.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

4.Kim K.W, Chung H.H, Chung C.W, Kim I.K, Miura M, Wang S, Zhu H, Moon K.D, Rha G.B, Park J.H, Jo D.G, Woo H.N, Song Y.H, Kim B.J, Yuan J, Jung Y.K: Inactivation of farnesyltransferase and geranylgeranyltransferase I by caspase-3: Cleavage of the common alpha subunit during apoptosis. Oncogene 20: 358-366, 2001

5.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

6.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

水澤英洋　（東京医科歯科大学・大学院医歯学総合研究科）

1.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

2.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

3.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

4.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

5.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 α-tocopherol transfer protein: Model for neuronal degeneration caused by chronic oxidative stress. Proc Natl Acad Sci USA 98: 15185-15190, 2001

森下竜一　（大阪大学・大学院医学系研究科）

1.Nakagami H, Morishita R, Yamamoto K, Taniyama Y, Aoki M, Matsumoto K, Nakamura T, Kaneda Y, Horiuchi M, Ogihara T: Mitogenic and anti-apoptotic actions of HGF through ERK, STAT3 and Akt in endothelial cells. Hypertension 37: 581-586, 2001

2.Taniyama Y, Morishita R, Aoki M, Nakagami H, Yamamoto K, Yamazaki K, Matsumoto K, Nakamura T, Kaneda Y, Ogihara T: Therapeutic angiogenesis induced by human hepatocyte growth factor gene in rat and rabbit hind limb ischemia models: preclinical study for treatment of peripheral arterial disease. Gene Ther 8:181-189, 2001

3.Tsuboniwa N, Morishita R, Hirano T, Fujimoto J, Furukawa S, Kikumori M, Okuyama A, Kaneda Y: Safety evaluation of hemagglutinating virus of Japan-artificial viral envelope liposomes in nonhuman primates. Hum Gene Ther 12: 469-487, 2001

4.Yamamoto K, Morishita R, Hayashi S, Matsushita H, Nakagami H, Moriguchi A, Matsumoto K, Nakamura T, Kaneda Y, Ogihara T: Contribution of Bcl-2. but not Bcl-xL and Bax, to anti-apoptotic actions of hepatocyte growth factor in hypoxic conditioned human endothelial cells. Hypertension 37: 1341-1348, 2001

5.Nakagami H, Morishita R, Yamamoto K, Yoshimura S, Taniyama Y, Aoki M, Matsubara H, Kim S, Kaneda Y, Ogihara T: Phosphorylation of p38MAP kinase downstream of bax-caspase-3 pathway leads to cell death induced by high D-glucose in human endothelial cells. Diabetes 50: 1472-1481, 2001

6.Kawamura I, Morishita R, Tsujimoto S, Manda T, Tomoi M, Tomita N, Goto T, Ogihara T, Kaneda Y: Intravenous injection of oligodeoxynucleotides to the NF-kappaB binding site inhibits hepatic metastasis of M5076 reticulosarcoma in mice. Gene Ther 8: 905-912, 2001

7.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

8.Ahn JD, Morishita R, Kaneda Y, Lee KU, Park JY, Jeon YJ, Song HS, Lee IK: Transcription factor decoy DON for activator protein-1 (AP-1 inhibits expression of type 1 plasminogen activator inhibitor (PAI-1) gene induced by high glucose and angiotensin II in cultured human vascular smooth muscle cells. Diabetologia 44: 713-720, 2001

9.Aoki M, Nata T, Morishita R, Matsushita H, Nakagami H, Yamamoto K, Yamazaki K, Nakabayashi M, Ogihara T, Kaneda Y: Endothelial apoptosis induced by oxidative stress through activation of NFkB: anti-apoptotic effect of antioxidant agents on endothelial cells. Hypertension 38: 48-55, 2001

10.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

11.Otomo T, Yamamoto S, Morishita R, Kaneda Y: EBV replicon vector system enhances transgene expression in vivo: applications to gene therapy for cancer. J Gene Med 3: 345-352, 2001

12.Sakaue G, Shimaoka M, Fukuoka T, Hiroi T, Inoue T, Hashimoto N, Sakaguchi T, Sawa Y, Morishita R, Kiyono H, Noguchi K, Mashimo T: NFkB decoy suppresses cytokine expression and thermal hyperalgesia in a rat neuropathic model. Somatosensory Systems Pain 12: 2079-2084, 2001

13.Aoki M, Morishita R, Hayashi SI, Jo N, Matsumoto K, Nakamaura T, Kaneda Y, Ogihara T: Inhibition of neointimal formation after balloon injury by cilostazol, accompanied by improvement of endothelial dysfunction and induction of hepatocyte growth factor in rat diabetes model. Diabetologia 44: 1034-1042, 2001

14.Ueno 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

15.Taniyama Y, Morishita R, Hiraoka K, Aoki M, Nakagami H, Yamasaki K, Matsumoto K, Nakamura T, Kaneda Y, Ogihara T: Therapeutic angiogenesis induced by human hepatocyte growth factor gene in rat diabetic hind limb ischemia model: molecular mechanisms of delayed angiogenesis in diabetes. Circulation 104: 2344-2350, 2001

16.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

17.Morishita R, Aoki M, Kaneda Y, Ogihara T: Gene therapy in vascular medicine: recent advances and future perspectives. Pharmacol Ther 91: 105-114, 2001

18.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 in press

19.Taniyama Y, Tachibana K, Hiraoka K, Aoki M, Yamamoto S, Matsumoto K, Nakamura T, Ogihara T, Kaneda Y, Morishita R: Development of safe and efficient novel non-viral gene transfer using ultrasound: enhancement of transfection efficiency of naked plasmid DNA in skeletal muscle. Gene Ther in press

20.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 in press

21.Nakamura T, Morishita R, Asai T, Tsuboniwa N, Aoki M, Sakonjo H, Yamasaki K, Hashiya N, Kaneda Y, Ogihara T: Molecular strategy using cis-element “decoy” of E2F binding site inhibits neointimal formation in porcine balloon-injured coronary artery model. Gene Ther in press

22.Morishita R, Sakaki M, Yamamoto K, Iguchi S, Aoki M, Yamasaki K, Matsumoto K, Nakamura T, Lawn R, Ogihara T, Kaneda Y: Impairment of collateral formation in Lp(a) transgenic mice: therapeutic angiogenesis induced by human hepatocyte growth factor gene. Circulation in press

吉澤利弘　（筑波大学・臨床医学系）

1.Yoshizawa T, Yoshida H, Shoji S: Differential susceptibility of cultured cells to aggregate formation and death by the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch. Brain Res Bull 56: 349-352, 2001

2.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

3.Hatakeyama S, Wakamori M, Ino M, Miyamoto M, Takahashi E, Yoshinaga T, Sawada K, Imoto K, Tanaka I, Yoshizawa T, Nishizawa Y, Mori Y, Niidome T, Shoji S: Differential nociceptive response in mice lacking the alpha1B subunit of N-type Ca²⁺ channels. NeuroReport 12: 2423-2427, 2001

4.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 in press

吉田裕樹　（九州大学・生体防御医学研究所）

1.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

2.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

若松延昭　（愛知県心身障害者コロニー・発達障害研究所）

1.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

2.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 (Online#443) 18: 253, 2001

3.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