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西村 正樹

所属部署名神経難病研究センター
職名教授
 
 
更新日: 18/03/30 10:27

研究者基本情報

氏名

    西村 正樹

所属(マスタ)

  • 神経難病研究センター 教授

学歴

  • - 1996年京都大学 医学研究科 内科系
  • - 1984年京都大学 医学部 医学科

学位

  • 医学博士(京都大学)

所属学協会

    日本神経病理学会 , 日本神経学会 , 日本分子生物学会 , 日本生化学会 , 日本神経科学会 , Society for Neuroscience , 日本痴呆学会

経歴

  • 2007年- 現在 滋賀医科大学 准教授
  • 2000年- 2007年 滋賀医科大学 助教授
  • 1997年- 1999年 トロント大学 博士研究員

研究活動情報

研究キーワード

    アルツハイマー病

論文

  • Neuronal expression of ILEI/FAM3C and its reduction in Alzheimer's disease.
    Liu L., Akatsu H., Watanabe N., Nishimura M.
    Neuroscience 330 236-246 2016年08月 [査読有り]
  • Generation of transgenic cynomolgus monkeys that express green fluorescent protein throughout the whole body.
    Itoh Y., Hitoshi S., Nishimura M., Imamura T., Miyoshi H., Tooyama I., Ogasawara K., Saitou M., Ema M., Sasaki E., Seita Y., Iwatani C., Tsukiyama T., Matsushita J., Tsuchiya H., Okahara J., Azami T., Hayashi Y., Nakamura S.
    Sci Rep 6 2016年04月 [査読有り]
  • Immunohistochemical detection of the delayed formation of nonfibrillar large amyloid-β aggregates.
    Ochiishi Tomoyo, Itakura Akira, Liu Lei, Akatsu Hiroyasu, Akatsu Hiroyasu, Kohno Hideki, Nishimura Masaki, Yoshimune Kazuaki
    Genes Cells 21(2) 211 2016年02月 [査読有り]
  • Presynaptic localization of the gamma-secretase-inhibiting protein p24alpha2 in the mammalian brain.
    Liu L., Nishimura M., Fujino K.
    J Neurochem 133(3) 422-431 2015年05月 [査読有り]
  • The FAM3 superfamily member ILEI ameliorates Alzheimer's disease-like pathology by destabilizing the penultimate amyloid-β precursor.
    Hasegawa H., Tooyama I., Liu L., Nishimura M., Murayama S.
    Nat Commun 5 2014年
  • OCIAD2 activates γ-secretase to enhance amyloid β production by interacting with nicastrin.
    HAN Jonghee, JUNG Sunmin, JANG Jiyeon, KAM Tae-In, CHOI Hyunwoo, KIM Byung-Ju, NAH Jihoon, JO Dong-Gyu, NAKAGAWA Toshiyuki, NISHIMURA Masaki, JUNG Yong-Keun
    Cell Mol Life Sci 71(13) 2561-2576 2014年
  • Membrane-microdomain localization of amyloid β-precursor protein (APP) C-terminal fragments is regulated by phosphorylation of the cytoplasmic Thr668 residue.
    Suzuki T., Matsushima T., Elliott J.I., Saito Y., Nishimura M., Iijima-Ando K., Hata S., Kimura N., Nakaya T., Yamamoto T.
    J Biol Chem 287(23) 19715-19724 2012年
  • Age-related modulation of γ-secretase activity in non-human primate brains.
    NISHIMURA Masaki, NAKAMURA Shin‐ichiro, KIMURA Nobuyuki, LIU Lei, SUZUKI Toshiharu, TOOYAMA Ikuo
    J Neurochem 123(1/2) 21-28 2012年
  • Hyperpolarization-activated cyclic nucleotide gated channels: a potential molecular link between epileptic seizures and Aβ generation in Alzheimer’s disease.
    Imoto K., Shigemoto R., Suzuki T., Saito Y., Zhu G., Inoue T., Okada M., Kimura N., Kimura N., Nishimura M., Kaneko S., Murayama S.
    Mol Neurodegener  7(1) 2012年
  • Alternative processing of γ-secretase substrates in common forms of mild cognitive impairment and Alzheimer’s disease: Evidence for γ-secretase dysfunction.
    Ann Neurol 2011年
  • Nuclear choline acetyltransferase activates transcription of a high-affinity choline transporter
    MATSUO Akinori, BELLIER Jean‐Pierre, NISHIMURA Masaki, YASUHARA Osamu, SAITO Naoaki, KIMURA Hiroshi
    Journal of Biological Chemistry 286(7) 5836-5845 2011年
  • Potent amyloidogenicity and pathogenicity of Aβ43.
    SAITO Takashi, SUEMOTO Takahiro, BROUWERS Nathalie, BROUWERS Nathalie, SLEEGERS Kristel, SLEEGERS Kristel, FUNAMOTO Satoru, MIHIRA Naomi, MATSUBA Yukio, YAMADA Kazuyuki, NILSSON Per, TAKANO Jiro, NISHIMURA Masaki, IWATA Nobuhisa, IWATA Nobuhisa, VAN BROECKHOVEN Christine, VAN BROECKHOVEN Christine, IHARA Yasuo, SAIDO Takaomi C
    Nat Neurosci 14(8) 1023-1032 2011年
  • Human CRB2 inhibits γ-secretase cleavage of amyloid precursor protein by binding to the presenilin complex.
    MITSUISHI Yachiyo, HASEGAWA Hiroshi, MATSUO Akinori, ARAKI Wataru, SUZUKI Toshiharu, TAGAMI Shinji, OKOCHI Masayasu, TAKEDA Masatoshi, ROEPMAN Ronald, NISHIMURA Masaki
    J Biol Chem 285(20) 14920-14931 2010年
  • Dilysine retrieval signal-containing p24 proteins collaborate in inhibiting γ-cleavage of amyloid precursor protein.
    HASEGAWA Hiroshi, LIU Lei, NISHIMURA Masaki
    J Neurochem 115(3) 771-781 2010年
  • Destruxin E decreases β-amyloid generation by reducing colocalization of β-amyloid-cleaving enzyme 1 and β-amyloid protein precursor.
    Hasegawa H., Nishimura M., Sakaguchi G., Kato A., Haass C., Morihara T., Tanaka T., Kudo T., Takeda M., Itoh N., Tagami S., Okochi M., Nakayama T., Nishitomi K., Fukumori A., Yanagida K., Mori K., Jiang J., Kikuchi J., Hosono M., Takinami Y., Nakano Y., Nishigaki A., Dohi K., Minagawa K., Takemoto H., Willem M., Katoh T.
    Neurodegener Dis 6(5-6) 230-239 2009年
  • Alcadein cleavages by APP α-and γ-secretases generate small peptides p3-Alcs indicating Alzheimer disease-related γ-secretase dysfunction.
    HATA Saori, FUJISHIGE Sayaka, ARAKI Yoichi, KATO Naoko, ARASEKI Masahiko, NISHIMURA Masaki, HARTMANN Dieter, SAFTIG Paul, FAHRENHOLZ Falk, TANIGUCHI Miyako, URAKAMI Katsuya, AKATSU Hiroyasu, MARTINS Ralph N., YAMAMOTO Kazuo, MAEDA Masahiro, YAMAMOTO Tohru, NAKAYA Tadashi, GANDY Sam, GANDY Sam, SUZUKI Toshiharu
    J Biol Chem 284(52) 36024-36033 2009年
  • γ-Secretase complex: core components and modulators.
    Recent Advances in the Biology of Secretases, Key Proteases in Alzheimer Disease 133-150 2008年
  • Reconstitution of gamma-secretase by truncated presenilin (PS) fragments revealed that PS C-terminal transmembrane domain is critical for formation of gamma-secretase complex
    SHIRAISHI Hirohisa, SHIRAISHI Hirohisa, MARUTANI Toshihiro, WANG Hua‐Qin, MAEDA Yasuhiro, KURONO Yukihisa, TAKASHIMA Akihiko, ARAKI Wataru, NISHIMURA Masaki, YANAGISAWA Katsuhiko, KOMANO Hiroto
    Genes Cells 11(1) 83-93 2006年
  • Random mutagenesis of presenilin-1 identifies novel mutants exclusively generating long amyloid beta-peptides
    NAKAYA Yoshifumi, YAMANE Takuya, SHIRAISHI Hirohisa, MATSUBARA Etsuro, SATO Toru, DOLIOS Georgia, DE STROOPER Bart, KOMANO Hiroto, FRASER Paul
    J Biol Chem 280(19) 19070-19077 2005年
  • Interaction of presenilins with FKBP38 promotes apoptosis by reducing mitochondrial Bcl-2
    WANG Hq, NAKAYA Y, DU Zy, YAMANE T, SHIRANE M, KUDO T, TAKEDA M, TAKEBAYASHI K, NODA Y, NAKAYAMA Ki, NISHIMURA M
    Hum Mol Genet 14(13) 1889-1902 2005年
  • Coordinated metabolism of Alcadein and amyloid beta-protein precursor regulates FE65-dependent gene transactivation
    ARAKI Y, MIYAGI N, KATO N, YOSHIDA T, NISHIMURA M, KOMANO H, YAMAMOTO T, DE STROOPER B, YAMAMOTO K
    J Biol Chem 279(23) 24343-24354 2004年
  • PEN-2 enhances gamma-cleavage after presenilin heterodimer formation
    SHIRAISHI H, SAI X, WANG H‐Q, MAEDA Y, KURONO Y, NISHIMURA M, YANAGISAWA K, KOMANO H
    J Neurochem 90(6) 1402-1413 2004年
  • Follistatin-related gene (FLRG) expression in human endometrium: sex steroid hormones regulate the expression of FLRG in cultured human endometrial stromal cells
    WANG Hq, TAKEBAYASHI K, TSUCHIDA K, NISHIMURA M, NODA Y
    J Clin Endocrinol Metab  88(9) 4432-4439 2003年
  • Normal brain development in PS1 hypomorphic mice with markedly reduced γ-secretase cleavage of betaAPP
    Schmidt S.D., Mathews P., Bergeron C., Mercken M., St George-Hyslop P., Westaway D., Rozmahel R., Chen F., Huang J., Nguyen V., Liang Y., Levesque G., Ikeda M., Nishimura M., Yu G.
    Neurobiol Aging  23(2) 187-194 2002年
  • Nicastrin binds to membrane-tethered Notch
    SATO C, YU C, JANUS C, LEE J, SONG Lx, ZHANG Ll, FRASER Pe, ST GEORGE-HYSLOP Ph, MILMAN P, ROGAEVA E, SONG Yq, CHEN Fs, YU G, ARAWAKA S, NISHIMURA M, KAWARAI T, YU H, TANDON A, SUPALA A
    Nat Cell Biol 3(8) 751-754 2001年
  • Evidence that the b-catenin nuclear translocation assay allows for measuring presenilin 1 dysfunction.
    Mol Med 6 570-580 2000年
  • Mutation of conserved aspartates affect maturation of both aspartate-mutant and endogenous presenilin 1 and presenilin 2 complex
    YU G, CHEN F, NISHIMURA M, TANDON A, KAWARAI T, SUPALA A, HOLMES E, FRASER P E, ST GEORGE‐HYSLOP P
    J Biol Chem 275(35) 27348-27353 2000年
  • Nicastrin modulates presenilin-mediated notch/glp-1 signal transduction and betaAPP processing
    YU G, LEVITAN D, ZHANG L, ZHANG Y, AEBERSOLD R, FARRER L, SORBI S, BRUNI A, ST GEORGE‐HYSLOP P
    Nature 407(6800) 48-54 2000年
  • Mutation of the conserved N-terminal cysteine (Cys92) of human presenilin 1 causes increased Ab42 secretion in mammalian cells but impaired Notch/lin-12 signalling in C. elegans.
    Neuroreport 11(14) 3227-3230 2000年
  • Carboxyl-terminal fragments of Alzheimer {beta}-amyloid precursor protein accumulate in restricted and unpredicted intracellular
    CHEN F, PETANCESKA S, YANG A, ROZMAHEL R, GHISO J, NISHIMURA M, LEVESQUE L, GANDY S, FRASER P E
    J Biol Chem 275(47) 36794-36802 2000年
  • Effects of antithrombin on Binswanger's disease with antiphospholipid antibody syndrome.
    Neurology 52(2) 398-401 1999年
  • Presenilin mutations associated with Alzheimer disease cause defective intracellular trafficking of b-catenin, a component of t
    NISHIMURA M, YU G, LEVESQUE G, ZHANG D M, RUEL L, CHEN F, ROZMAHEL R, MATTILA K, ST GEORGE‐HYSLOP P
    Nature Med 5(2) 164-169 1999年
  • Presenilins interact with armadillo proteins including neural-specific plakophilin-related protein and b-catenin.
    LEVESQUE G, YU G, NISHIMURA M, ZHANG D M, LEVESQUE L, YU H, LIANG Y, ROGAEVA E, GEORGE‐HYSLOP P ST
    J Neurochem 72(3) 999-1008 1999年
  • Multifocal polyradiculoneuropathy and carcinoma of the thymus.
    OHTAKE T, NISHIMURA M, ODA M
    J Neurol Sci 168(1) 62-67 1999年
  • An -alfa-2-macroglobulin insertion-deletion polymorphism in Alzheimer disease
    Nature Genet  22(1) 19-20 1999年
  • Analysis of the butyrylcholinesterase gene and nearby chromosome 3 markers in Alzheimer disease.
    Duara R., Sorbi S., St George-Hyslop P., Farrer L., Brindle N., Rogaeva E., Song Y., Levesque G., Premkumar S., Ikeda M., Yu G., Paterson A., Nishimura M.
    Hum Mol Genet 7(5) 933-935 1998年
  • The presenilin 1 protein is a component of a high molecular weight intracellular complex that contains b-catenin.
    YU G, CHEN F, LEVESQUE G, NISHIMURA M, LEVESQUE L, ROGAEVA E, XU D, ST GEORGE‐HYSLOP P H, FRASER P E
    J Biol Chem 273(26) 16470-16475 1998年
  • Absence of association between Alzheimer disease and the -491 regulatory region polymorphism of APOE.
    SONG Y‐Q, YU G, IKEDA M, PEI Y, DUARA R, SORBI S, FREEDMAN M, FARRER L, ST GEORGE‐HYSLOP P
    Neurosci Lett 250(3) 189-192 1998年
  • Evidence for an Alzheimer disease susceptibility locus on chromosome 12 and for further locus heterogeneity.
    ROGAEVA E, SORBI S, DUARA R, YU G, IKEDA M, JORGE R, BRUNI A C, FARRER L A, ST. GEORGE‐HYSLOP P H
    JAMA 280(7) 614-618 1998年
  • Large neurons in the tuberomammillary nucleus in patients with Parkinson's disease and multiple system atrophy.
    Neurology 46(6) 1693-1696 1996年
  • Alterations of the blood-brain barrier and glial cells in white-matter lesions in cerebrovascular and Alzheimer's disease patien
    Stroke 27(11) 2069-2074 1996年
  • Immunocytochemical characterization of glial fibrillary tangles in Alzheimer's disease brain.
    Am J Pathol 146(5) 1052-1058 1995年
  • Bundles of amyloid precursor protein-immunoreactive axons in human cerebrovascular white matter lesions.
    Acta Neuropathol 87(5) 450-455 1994年
  • Synaptophysin and chromogranin A immunoreactivities of Lewy bodies in Parkinson's disease brains.
    NISHIMURA M, TOMIMOTO H, SUENAGA T, NAKAMURA S, NAMBA Y, IKEDA K, AKIGUCHI I, KIMURA J
    Brain Res 634(2) 339-344 1994年
  • Corticobasal degeneration: a disease with widespread appearance of abnormal tau and neurofibrillary tangles, and its relation t
    Acta Neuropathol 88(2) 113-121 1994年
  • Distribution of nitric oxide synthase in the human cerebral blood vessels and brain tissues.
    J Cereb Blood Flow Metab 14(6) 930-938 1994年
  • CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1.
    KAWAGUCHI Y, TANIWAKI M, AIZAWA M, KATAYAMA S, KAWAKAMI H, NAKAMURA S, NISHIMURA M, KIMURA J, KAKIZUKA A
    Nature Genet 8(3) 221-228 1994年
  • GAP-43 immunoreactivity is detected in the nerve terminals of patients with amyotrophic lateral sclerosis.
    Ann Neurol 33(2) 226-227 1993年
  • Neurofibrillary tangles in the neurons of spinal dorsal root ganglia of patients with progressive supranuclear palsy.
    Acta Neuropathol 85(5) 453-457 1993年
  • Loss of large neurons and occurrence of neurofibrillary tangles in the tuberomammillary nucleus of patients with Alzheimer's dis
    NAKAMURA S, TAKEMURA M, OHNISHI K, SUENAGA T, NISHIMURA M, AKIGUCHI I, KIMURA J, KIMURA T
    Neurosci Lett 151(2) 196-199 1993年
  • Abnormal accumulation of phospholipase C-d in filamentous inclusions of human neurodegenerative diseases.
    Neurosci Lett 162(1-2) 183-186 1993年
  • Immunohistochemical expression of microtubule-associated protein 5 (MAP5) in glial cells in multiple system atrophy.
    J Neurol Sci 109(1) 102-106 1992年
  • Glial fibrillary tangles with straight tubules in the brains of patients with progressive supranuclear palsy.
    Neurosci Lett 143(1-2) 35-38 1992年
  • A necrobiotic form of astrocyte in non-missile head injury - an immunohistochemical study - .
    Neuropathol 12 47-50 1992年
  • Cerebral angio- and neuro-Behcet's syndrome: neuroradiological and pathological study of one case.
    J Neurol Sci 106(1) 19-24 1991年
  • Chronic progressive spinobulbar spasticity with disturbance of voluntary eyelid closure. Report of a case with special reference
    J Neurol Sci 96(2-3) 183-190 1990年

受賞

  • 1999年 Frist/Jus記念賞(Frist/Jus Memorial Prize)

競争的資金

  • アルツハイマー病における神経変性の分子機構に関する研究
    :科学研究費補助金