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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: 17/09/20 14:37

研究者基本情報

氏名

    西村 正樹

基本情報

    ハンドル:  handle0064576
    科研費研究者番号:  40322739

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.
    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.
    Sci Rep 6 24868 2016年04月 [査読有り]
  • Immunohistochemical detection of the delayed formation of nonfibrillar large amyloid-β aggregates.
    Genes Cells 21(2) 200-211 2016年02月 [査読有り]
  • Presynaptic localization of the gamma-secretase-inhibiting protein p24alpha2 in the mammalian brain.
    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.
    Nat Commun 5 3917 2014年
  • OCIAD2 activates γ-secretase to enhance amyloid β production by interacting with nicastrin.
    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.
    J Biol Chem 287(23) 19715-19724 2012年
  • Age-related modulation of γ-secretase activity in non-human primate brains.
    J Neurochem 123(1) 21-28 2012年
  • Hyperpolarization-activated cyclic nucleotide gated channels: a potential molecular link between epileptic seizures and Aβ generation in Alzheimer’s disease.
    Mol Neurodegener  7 50 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
    Journal of Biological Chemistry 286(7) 5836-5845 2011年
  • Potent amyloidogenicity and pathogenicity of Aβ43.
    Nat Neurosci 14(8) 1023-1032 2011年
  • Human CRB2 inhibits γ-secretase cleavage of amyloid precursor protein by binding to the presenilin complex.
    J Biol Chem 285(20) 14920-14931 2010年
  • Dilysine retrieval signal-containing p24 proteins collaborate in inhibiting γ-cleavage of amyloid precursor protein.
    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.
    Neurodegener Dis 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.
    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
    Genes Cells 11(1) 83-93 2006年
  • Random mutagenesis of presenilin-1 identifies novel mutants exclusively generating long amyloid beta-peptides
    J Biol Chem 280(19) 19070-19077 2005年
  • Interaction of presenilins with FKBP38 promotes apoptosis by reducing mitochondrial Bcl-2
    Hum Mol Genet 14(13) 1889-1902 2005年
  • Coordinated metabolism of Alcadein and amyloid beta-protein precursor regulates FE65-dependent gene transactivation
    J Biol Chem 279(23) 24343-24354 2004年
  • PEN-2 enhances gamma-cleavage after presenilin heterodimer formation
    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
    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
    Neurobiol Aging  23(2) 187-194 2002年
  • Nicastrin binds to membrane-tethered Notch
    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
    J Biol Chem 275 27348-27353 2000年
  • Nicastrin modulates presenilin-mediated notch/glp-1 signal transduction and betaAPP processing
    Nature 407 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
    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
    Nature Med 5(2) 164-169 1999年
  • Presenilins interact with armadillo proteins including neural-specific plakophilin-related protein and b-catenin.
    J Neurochem 72 999-1008 1999年
  • Multifocal polyradiculoneuropathy and carcinoma of the thymus.
    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.
    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.
    J Biol Chem 273(26) 16470-16475 1998年
  • Absence of association between Alzheimer disease and the -491 regulatory region polymorphism of APOE.
    Neurosci Lett 250(3) 189-192 1998年
  • Evidence for an Alzheimer disease susceptibility locus on chromosome 12 and for further locus heterogeneity.
    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.
    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.
    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
    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