辻　省次（東京大学・大学院医学系研究科）

1.Iwata A, Maruyama M, Akagi T, Hashikawa T, Kanazawa I, Tsuji S et al.: Alpha-synuclein degradation by serine protease neurosin: Implication for pathogenesis of synucleinopathies. Hum Mol Genet 12: 2625-2635, 2003

2.Sanpei K, Ikeuchi T, Tsuji S: DIRECT technologies for molecular cloning of genes containing expanded CAG repeats. Meth Mol Biol 217: 73-81, 2003

3.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

4.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

5.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

6.Hara K, Fukushima T, Suzuki T, Shimohata T, Oyake M, Ishiguro H, Hirota K, Miyashita A, Kuwano R, Kurisaki H, Yomono H, Goto J, Kanazawa I, Tsuji S: Japanese SCA families with a distinct phenotype linked to a locus overlapping with SCA15 locus Neurology 62: 648-651，2004

岩坪　威（東京大学・大学院薬学系研究科）

1.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

2.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

3.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 Aß42 generation. J Biol Chem 278: 18664-18670, 2003

4.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

5.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

6.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

7.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

8.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

9.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

10.Taniguchi S, McDonagh AM, 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

11.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 in press

12.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 in press

13.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. Mol Cell Biol in press

14.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 in press

祖父江　元（名古屋大学・大学院医学系研究科）

1.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

2.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

3.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

4.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

5.Wada M, Kimura M, Daimon M, Kurita K, Kato T, Johmura Y, Johkura K, Kuroiwa Y, Sobue G: An usual phenotype of McLeod syndrome with late onset axonal neuropathy. J Neurol Neurosurg Psychiat 74: 1697-1698, 2003

6.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

7.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

8.Hishikawa N, Hashizume Y, Yoshida M, Sobue G: Clinical and neuropathological correlates of Lewy body disease. Acta Neuropathol 105: 341-350, 2003

9.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 and 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

10.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

11.Ishihara K, Yamagishi N, Saito Y, Adachi H, Kobayashi Y, Sobue G, Otsuka K, Hatayama T: Hsp 105 a suppresses the aggregation of truncated androgen receptor with expanded CAG repeats and cell toxicity. J Biol Chem 278: 25143-25150, 2003

12.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

13.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

14.Nodera H, Bostock H, Kuwabara S, Sakamoto T, Asamura 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

15.Watanabe H, Fukatsu H, Katsuno M, Sugiura M, Hamada K, Okada Y, Hirayama M, Ishigaki 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, 2003

16.Katsuno M, Adachi H, Sobue G: Sweet relief for Huntington’s disease. Nat Med 10: 123, 2004

17.Takeuchi H, Niwa J, Hishikawa N, Ishigaki S, Tanaka F, Doyu M, Sobue G: Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis. J Neurochem in press

18.Koike H, Misu K, Sugiura S, Iijima M, Mori K, Yamamoto M, Hattori N, Mukai E, Ando Y, Ikeda S, Sobue G: Pathologic differences between early-and late-onset type I (TTR Met30) familial amyloid polyneuropathy. Neurology in press

19.Katsuno M, Sobue G: Polyglutamine diminishes VEGF: Passage to motor neuron death? Neuron in press

20.Katsuno M, Adachi H, Tanaka F, Sobue G: Spinal and bulbar muscular atrophy (SBMA): Ligand-dependent pathogenesis and therapeutic perspective. J Mol Med in press

水野美邦（順天堂大学・医学部）

1.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

2.Gouider-Khouja Ｎ，Larnaout A, Amouri R, Sfar S, Belal S, Hamida C. B, Hamida M. B, 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

3.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

4.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

5.Takubo H, Shimoda-Matsubayashi S, 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

6.Okuma Y, Hattori N, Mizuno Y: case report, Sensory neuropathy in autosomal recessive juvenile parkinsonism (PARK2). Parkinsonism Relat Disord 9: 313-314, 2003

7.Ikebe S. Harada T, Hashimoto T, Kanazawa I, Kuno S, Mizuno Y, Mizuta E, Murata M, Nagatsu T, nakamura S, Takubo S, Yanagisawa N, Narabayashi H: Prevention and treatment of malignant syndrome in Parkinson's disease: a consensus statement of the malignant syndrome research group. Parkinsonism Relat Didord 9 (Suppl 1): 47-49, 2003

8.Inzelberg R, Hattori N, Nisipeanu P, Abo Mouch S, Blumen SC, Carasso R. L., Mizuno Y: Camptocoemia, axial dystonia, and parkinsonism: Phenotypic heterogeneity of a parkin mutation. Neurology 60: 1393-1394, 2003

9.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

10.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

11.Kobayashi H, Kruger R, Markopoulou K, Wszolek Z, Chase B, Taka H, Mineki R, 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

12.Tanaka R, Komine-Kobayashi M, Mochizuki H, 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

13.Furuya T, Tanaka R, Urabe T, Hayakawa J, Migita M, Shimada T, Mizuno Y, Mochizuki H: Establishment of modified cyhimeric mice using GFP bone marrow as a model for neurological disorders. NeuroReport 13: 1-3, 2003

14.Hatano T, Okuna Y, Iiijima M, Fujishima K, Goto K, Mizuno Y: Cervical dystonia in detatorubral-pallidoluysian atrophy. Acta Neurol Scand 108: 287-289, 2003

青木正志（東北大学・医学部附属病院神経内科）

1.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, T, Miyachi T, Yamashita H, Kawai M, Matsumura T, Kuzuhara S, Ibi T, Sahashi K, Nakai H, Kohnosu T, Nonaka I, Arahata K, Brown RH Jr, Saito H, Itoyama Y: Dysferlin mutations in Japanese Miyoshi myopathy: Relationship to phenotype. Neurology 60: 1799-1804, 2003

2.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

3.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 superxide 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

4.Suzuki N, Aoki M, Takahashi T, Takano D, Asano M, Shuga Y, Onodera Y, Tateyama M, Itoyama Y: Novel dysferlin mutations and characteristic muscle atrophy in late onset Miyoshi myopathy. Muscle Nerve in press

5.Yasuda H, Ebihara S, Aoki M, Yamaya M, Asada M, Sasaki H: Increased arterial carboxyhemoglobin concentraions in patients with sporadic amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiat in press

6.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 E3 Ubiquitin ligase for dishevelled1, targets mutant superoxide dismutase 1. J Biol Chem in press

青崎敏彦（東京都老人総合研究所）

1.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

五十嵐修一（新潟大学・脳研究所付属生命科学リソース研究センター）

1.Igarashi S, Morita H, Bennett KM, Tanaka Y, Engelender S, Peters MF, Cooper JK, Wood JD, Sawa A, Ross CA: Inducible PC12 cell model of Huntington's disease shows toxicity and decreased histone acetylation. NeuroReport 14: 565-568, 2003

2.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

石川欽也（東京医科歯科大学・医学部附属病院）

1.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

2.Kubodera T, Yokota T, Owada K, Ishikawa K, Miura H, Matsuoka T, Mizusawa H: Proteolytic cleavage and cellular toxixity of the human alpha1A calcium channel in spinocerebellar ataxia type 6. Neurosci Lett 341: 74-78, 2003

3.Yashima T, Noguchi Y, Ishikawa K, Mizusawa H, Kitamura K: Mutation of the EYA1 gene in patients with Brachio-oto syndrome. Acta Otolaryngol 123: 279-282, 2003

4.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 in press

大野耕策（鳥取大学・医学部附属病院）

1.Matsuda J, Suzuki O, Oshima M, Yamamoto Y, Noguchi A, Takimoto K, Itoh M, Matsuzaki Y, Yasuda Y, Ogawa S, Sakata Y, Nanba E, Higaki K, Ogawa Y, Tominaga L, Ohno K, Iwasaki H, Watanabe H, Brady RO, Suzuki Y: Chemical chaperone therapy for brain pathology in GM1-gangliosidosis. Proc Natl Acad Sci USA 100:15912-15917, 2003

2.Iwakuma M, Anzai T, Kobayashi S, Ogata M, Kaneda Y, Ohno K, Saji M: Antisense in vivo knockdown of synaptotagmin I and synapsin I by HVJ-liposome mediated gene transfer modulates ischemic injury of hippocampus in opposing ways. Neurosci Res 45: 285-296, 2003

3.Ono H, Sakura N, Yamashita K, Yuasa I, Ohno K: Novel nonsense mutation (R194X) in the PMM2 gene in a Japanese patient with congenital disorder of glycosylation type Ia. Brain Dev 25: 525-528, 2003

4.Matsuzawa F, Aikawa SI, Sakuraba H, Lan HT, Tanaka A, Ohno K, Sugimoto Y, Ninomiya H, Doi H: Structural basis of the GM2 gangliosidosis B variant. J Hum Genet 48: 582-589, 2003

郭　伸（東京大学・大学院医学系研究科）

1.Kawahara Y, Kwak S, Sun H, Ito K, Hashida H, Aizawa H, Jeong S-Y, Kanazawa I: Human spinal motoneurons express low relative abundance of GluR2 mRNA: An implication for excitotoxicity in ALS. J Neurochem 85: 680-689, 2003

2.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

3.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

4.Kawahara Y, Ito K, Sun H, Ito M, 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

5.Kawahara Y, Ito K, Sun H, Aizawa H, Kanazawa I, Kwak S: RNA editing and death of motor neurons. Nature 427: 801, 2004

嘉村　巧（九州大学・生体防御医学研究所）

1.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

2.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

3.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

木村洋子（東京都臨床医学総合研究所・腫瘍細胞研究部門）

1.Kimura Y, Koitabashi S, Fujita T: Analysis of yeast prion aggregates with amyloid-staining compound in vivo. Cell Struc Func 28: 187-193, 2003

坂口末廣（長崎大学・大学院医歯薬学総合研究科）

1.Watarai M, Kim S, Erdenebaatar J, Makino S, Horiuchi M, Shirahata T, Sakaguchi S, Katamine S: Cellular prion protein promotes Brucella infection into macrophages. J Exp Med 198: 5-17, 2003

2.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

3.Sakudo A, Lee D, Yoshimura E, Nagasaka S, Nitta K, Saeki K, Matsumoto Y, Lehmann S, Itohara S, Sakaguchi S, and Onodera T: Prion protein suppresses perturbation of cellular copper homeostasis under oxidative conditions. Biochem Biophys Res Commun 313: 845-850, 2004

佐々木秀直（北海道大学・大学院医学研究科）

1.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

2.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

3.Yabe I, Sasaki H, Chen H-H, Raskind WH, Bird TD, Yamashita I, Tsuji S, Tashiro K: Spinocerebellar ataxia type 14 caused by a mutation in protein kinase C gamma. Arch Neurol 60: 1749-1751, 2003

柴田昌宏（大阪大学・大学院医学系研究科）

1.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 (BACE). J Biol Chem 278: 15239-15245, 2003

2.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-b1 (TGF-beta1). J Neurosci Res 72: 33-45, 2003

3.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

4.Shiba T, Kametaka S, Kawasaki M, Shibata M, Waguri S, Uchiyama Y, Wakatsuki S: Insights into the phosphoregulation of beta-secretase sorting signal by the VHS domain of GGA1. Traffic in press

鈴木俊顕（東京都臨床医学総合研究所）

1.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 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）

1.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

2.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

3.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

4.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

5.Yokota T, Sugawara K, Ito 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

6.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

7.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

8.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 in press

田邊　勉（東京医科歯科大学・大学院医歯学総合研究科）

1.Kurihara T, Nonaka T, Tanabe T: Acetic acid conditioning stimulus induces long-lasting antinociception of somatic inflammatory pain. Pharmacol Biochem Behav 74: 841-849, 2003

2.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 Analg 97: 96-103, 2003

3.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 Lett 350: 41-45, 2003

4.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

程　久美子（東京大学・大学院理学系研究科）

1.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

2.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

3.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

4.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. Nuc Acids Res 32: 936-948, 2004

5.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 Biologiya in press

永井義隆（大阪大学・大学院医学系研究科）

1.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

中村浩一郎（東京大学・大学院医学系研究科）

1.Gao R, Coolbaugh M, Zülke C, Nakamura K, Rasmussen A, Matsuura T, Siciliano M, Ashizawa T, Lin X. Instability of expanded CAG/CAA repeats in spinocerebellar ataxia type 17. Hum Mol Genet in press

貫名信行（理化学研究所・脳科学総合研究センター）

1.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

2.Zemskov EA, Nukina N: Impaired degradation of PKCalpha by proteasome in a cellular model of Huntington's disease. NeuroReport 14: 1435-1438, 2003

3.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

4.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

5.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

6.Lee JA, Lim CS, Lee SH, 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

7.Tanaka M, Machida Y, Niu S, Ikeda T, Jana NR, Doi H, Kurosawa M, Nekooki M, Nukina N: Trehalose effectively alleviates polyglutamine-mediated pathology in a transgenic mouse model of Huntington's disease. Nat Med 10: 148-154, 2004

長谷川一子（北里大学・医学部）

1.Y Mizuno, N Yanagisawa, S Kuno, M Yamamoto, K Hasegawa, H Origasa, H Kowa, and 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

2.Hasegawa K, Funayama M, Kowa H, Tsuji S, Obata: Park 8 - new locus for an autosomal dominant Japanese family. ICN in press

久永眞市（東京都立大学・大学院理学研究科）

1.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

2.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

3.Honma N, Asada A, Takeshita S, Enomoto M, Yamakawa E, Tsutsumi K, Saito T, Sato T, Itoh H, Kaziro K, Kishimoto T, Hisanaga S: Apoptosis-associated tyrosine kinase (AATYK) is a Cdk5 activator p35 binding protein. Biochem Biophys Res Commun 310: 398-404, 2003

4.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

5.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

6.Uchida A, Tashiro T, Komiya Y, Yorifuji H, Kishimoto T, Hisanaga S: Neurofilament of aged rats: selective decrease in protein synthesis and the rate of axonal transport of neurofilament-m subunit. J Neurochem 88: 735-745, 2004

船越　洋（大阪大学・大学院医学系研究科）

1.Kato S, Funakoshi H, Nakamura T, Kato M, Nakano I, Hirano A, Ohama E: Expression of hepatocyte growth factor and c-Met in the anterior horn cells of the spinal cord in the patients with amyotrophic lateral sclerosis (ALS): immunohistochemical studies on sporadic ALS and familial ALS with superoxide dismutase 1 gene mutation. Acta Neuropathol 106: 112-120, 2003

2.Funakoshi H, Nakamura T: growth factor: from diagnosis to clinical applications. Clin Chim Acta 327: 1-23, 2003

3.Takahashi H, Funakoshi H, Nakamura T: LIM-kinase as a regulator of actin dynamics in spermatogenesis. Cytogen Genome Res in press

堀江秀典（早稲田大学・先端バイオ研究所）

1.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 Neuropath Exp Neurol 62: 162-172, 2003

2.Tahara K, Tsuchimoto D, Tominaga Y, Asoh S, Ohta S, Kitagawa M, Horie H, KadoyaT, and Nakabeppu Y: △FosB induces the delayed apoptosis independent of cell proliferation of the Rat1a embryo cell line. Cell Death Differ 10: 496-507, 2003

3.Imbe H, Okamoto K, Kadoy 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

4.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

5.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

6.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. Neuroscience in press

三浦正幸（東京大学・大学院薬学系研究科）

1.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

2.Kanuka H, Kuranaga E, Hiratou T, Igaki T, Nelson B, Okano H, Miura M: Cytosol-ER interplay by Sec61a translocon in polyglutamine-mediated neurotoxicity in Drosophila. Proc Natl Acad Sci USA 100: 11723-11728, 2003

3.Inoue H, Tsukita K, Iwasato T, Suzuki Y, Tomioka M, Tateno M, Nagao M, Kawata A, Saido TC, Miura M, 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

4.Iwawaki T, Akai R, Kohno K, Miura M: A transgenic mouse model for monitoring endoplasmic reticulum stress. Nat Med 10: 98-102, 2004

5.Furuya T, Hayakawa H, Yamada, M, Yoshimi K, Hisahara S, Miura M, Yoshikuni 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

水澤英洋（東京医科歯科大学・大学院医歯学総合研究科）

1.Miura Y, Misawa N, Inagaki Y, Yamatoto N, Ito M, Yagita H, Okumura K, Mizusawa H, Koyanagi Y: TNF-related apoptososis-inducing ligand on HIV-1-infected macrophages induces neuronal apoptosis in murine model of HIV encephalopathy. Proc Natl Acad Sci USA 100: 2777-2782, 2003

2.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

3.Furukawa Y, Hashimoto N, Yamakuni T, Ishida Y, Kato C, Ogashiwa M, Kobayashi M, Kobayashi T, Nonaka I, Mizusawa H, Song S: Down-regulation of an ankyrin repeat-containing protein, V-1, during skeletal muscle differentiation and its re-expression in the regenerative process of muscular dystrophy. Neuromusc Disord 13: 32-41, 2003

4.Kanda T, Yamawaki M, Mizusawa H: Sera from Guillain-Barré patients enhance leakage in blood-nerve barrier model. Neurology 60: 301-306, 2003

5.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: 111-113, 2003

6.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

7.Miura Y, Kobayashi Y, Mizusawa H: TNF-related apoptosis-inducing ligand (TRAIL) induces neuronal apoptosis in HIV-encephalopathy. J Med Dent Sci 50: 17-25, 2003

8.Ishibashi S, Kuroiwa T, Endo S, Okeda R, Mizusawa H: Neurological dysfunction versus regional infarction volume after focal ischemia in mongolian gerbils. Stroke 34: 1501-1506, 2003

9.Ishibashi S, Yokota T, Shiojiri T, Matunaga T, Tanaka H, Nishina K, Hirota H, Inaba A, Yamada M, Kanda T, Mizusawa H: Reversible acute axonal polyneuropathy associated with Wernicke-Korsakoff syndorome: impaired physiological nerve conduction due to thiamine deficiency? J Neurol Neurosurg Psychiat 74: 674-676, 2003

10.Yamada M, Sodeyama N, Itoh Y, Takahashi A, Matsushita M, Mizusawa H: Association of neprilysin polymorphism with cerebral amyloid angiopathy. J Neurol Neurosurg Psychiat 74: 749-751, 2003

11.Moinul Hossain AKM, Murata Y, Zhang L, Taura S, Saitoh Y, Mizusawa H, Oda K, Matsushita E, Okubo Y, Shibuya H: Brain perfusion SPECT in patients with corticobasal degeneration: analysis using statistical parametric mapping. Mov Disord 18: 697-703, 2003

12.Suzuki Y, Kiyosawa M, Ohno N, Mochizuki M, Inaba A, Mizusawa H, Ishii K, Senda M: Glucose hypometabolism in medial frontal cortex of patients with apraxia of lid opening. Clin Invest 241: 529-534, 2003

13.Suzuki Y, Kiyosawa M, Ohno N, Mochizuki M, Inaba A, Mizusawa H, Ishii K, Senda M: Glucose hypometabolism in medial frontal cortex of patients with apraxia of lid opening. Graefe's Arch Clin Exp Ophthalmol 241: 529-534, 2003

14.Yokota T, Sakamoto N, Enomoto N, Tanabe Y, Miyagishi M, Maekawa S, Yi L, Kurosaki M, Taira K, Watanabe M, Mizusawa H: Inhibition of intracellular hepatitis C virus replication by synthetic and vector-derived small interfering RNAs. EMBO Rep 4: 602-608, 2003

15.Yashima T, Noguchi Y, Ishikawa K, Mizusawa H, Kitamura K: Mutation of the EYA1 gene in patients with Brachio-oto syndrome. Acta Otolaryngol 123:279-282, 2003

16.Yokota T, Nishiwaki Y, Hiraoka M, Miyagishi M, Taira K, Isobe M, Mizusawa H, Yoshida M: Introduction of short interfering RNA to silence endogenous E-selectin in vascular endothelium leads to successful inhibition of leukocyte adhesion. Biochem Biophys Res Commun 310:1062-1066, 2003

17.Yokota T, Sugawara K, Ito K, Takahashi R, Ariga H, Misusawa 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

18.Ohkubo T, Sakasegawa Y, Asada T, Kinoshita T, Goto Y, Kimura H, Mizusawa H, Hachiya NS, Kaneko K: Absecne of associtation between codon 129/219 polymorphisms of the prion protein gene and Alzheimer’s disease in Japan. Ann Neurol 54: 553-554, 2003

19.Saito F, Tomimitsu H, Araki K, Kanda T, Mizusawa H, Shimizu T, Matsumoto K: A Japanease patient with distal myopathy with rimmed vacules: Missense mutations in the epimerase domain of the UDP-N-acetylglucosamine 2-epimerase/ N-acethylmannosamide kinase gene accompanied by hyposialylation of skeletal muscle glycoproteines. Neuromusc Disord in press

柳　茂（神戸大学・大学院医学系研究科）

1.Takahashi S, Inatome R, Hotta A, Qin Q, Hackenmiller R, Simon MC, Yamamura H, Yanagi S: Role for Fes/Fps tyrosine kinase in microtubule nucleation through its Fes/CIP4 homology domain. J Biol Chem 278: 49129-49133, 2003

山田光則（新潟大学・脳研究所・病理学分野）

1.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