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OHNO Kousaku  大野 耕策

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… Alternative Names

大野 耕策

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Researcher Number 70112109
External Links
Affiliation (based on the past Project Information) *help 2008 – 2010: Tottori University, 医学部, 教授
1993 – 2006: Tottori University Faculty of medicine, Department of Neurobiology, Professor, 医学部, 教授
2004: 国立大学法人鳥取大学, 医学部, 教授
1992: 鳥取大学, 医学部, 助教授
1986: 鳥取大学, 医学部, 講師
Review Section/Research Field
Principal Investigator
Biological Sciences / Pediatrics / Pediatrics
Except Principal Investigator
Pediatrics / Neurology / 応用薬理学・医療系薬学 / Cognitive science / Biological Sciences / Laboratory medicine
Keywords
Principal Investigator
ニーマン・ピック病C型 / 神経変性 / コレステロール / 遺伝病 / 糖鎖 / 糖蛋白 / dehydrodolichol reductase / asiolotransferrin / disialotransferrion / carbohydrate-deficient glycoprotein syndrome … More / ニーマン・ピック病 / リソゾーム / ゴーシェ病 / STAT / タウオパチー / ライソゾーム病 / glia / IL-6 / Toll-like receptor / cholesterol / Niemann-Pick disease C / N-octyl-β-valienamine / Chemical chaperone therapy / Gaucher disease / βグルコシダーゼ / 酵素蛋白質の安定化 / ニーマンピック病 / 小児 / 変異酵素活性化療法 / 分子シャペロン療法 / 分子シャペロン / asialotransferrin / disialotransferrin / dolichol / b-hexosaminidase / lysosome / transferrin / sugar chain / carbohydrate-deficient glycoprotein (CDG) / リソソーム酸素 / Carbohydrate-deficient glycoprotein / 先天性N-結合型糖鎖欠乏症候群 / 小脳失調 / ドリコール脂質中間1本 / 小脳** / ドリコール脂質中間体 / CDG症候群 / Carbohydrate-deficient / ドリコール / 小胞体 / immotalized cell / gene cloning / selectable markers / cholesterol transport / Niemann-Pick Desease / 染色体マッピング / 細胞内転送 / 薬剤感受性 / 遺伝子クローニング / 細胞内輸送 / モデルマウス / 経口治療薬 / シャペロン / サイトカイン / JAK / DNAマイクロアレイ / 小児期アルツハイマー病 / ガングリオシド / コレラトキシン / 小胞輸送 / 早期エンドソーム / 細胞内脂質輸送 / 神経遺伝病 / 神経細胞移植 / 神経前駆細胞 / 遺伝子治療 … More
Except Principal Investigator
LDL / コレステロール / Dementia / Developmental delay / Synapsis / Dendrite / Neuron / 脳低酸素症 / ニユーロン / 脳発達 / 痴呆 / Dovn症候群 / 発達遅滞 / シナプシス / 樹状突起 / 神経細胞 / secretase / Cholesterol / Amyloid β protein(Aβ) / Amyloid R protein precursor(APP) / Alzheimer's disease(AD) / アルミニウム / ニーマン・ピック病C型 / アミロイドβ蛋白(Aβ) / リノール酸 / アミロイドβタンパク (Aβ) / α-セレクターゼ / α-セクレターゼ / アミロイドβタンパク(Aβ) / APP遺伝子 / アルツハイマー病 / lysosomal disease / N-octyl-4-epi-β-valianamine / genetically engineered mouse / competitive inhibitor / G_<M1>-gangliosidosis / β-galactosidase / chemical chaperone therapy / GM1-ガングリオシドーシス / β-ガラクトシダーセ / ライソゾーム病 / N-オクチル-4-エピ-β-バリエナミン / 遺伝子組換えモデルマウス / 競合的阻害剤 / G_<M1>-ガングリオシドーシス / β-ガラクトシダーゼ / ケミカルシャペロン療法 / phosphomannomutase / antithrombin / transferrin / beta-hexosaminidase / alpha-fucosidase / CDG syndrome / 先天性代謝異常 / 糖蛋白質 / オリゴ糖 / α-フコシダーゼ / ホスホマンノムターゼ / アンチトロンビン / β-ヘキソサミニダーゼ / トランスフェリン / α、フコシダーゼ / CDG症候群 / 言語 / 学習 / 脳血流 / 視線計測 / 共同注意 / 機能的MRI / 対面コミュニケーション / 心の理論 / アストロサイト / サイトカイン / ニーマン・ピック / ヒトリポ蛋白 / ヒト血清 / ヒト血漿 / ヒト赤血球 / ヒト血漿リポ蛋白 / 低温培養細胞接着率 / 低温肝細胞培養 / 氷結点 / 氷温保存 Less
  • Research Projects

    (17 results)
  • Research Products

    (57 results)
  • Co-Researchers

    (36 People)
  •  Basic research for clinical treatment of neuropathic Gaucher disease by chemical chhaperonesPrincipal Investigator

    • Principal Investigator
      OHNO Kousaku
    • Project Period (FY)
      2008 – 2010
    • Research Category
      Grant-in-Aid for Scientific Research (B)
    • Research Field
      Pediatrics
    • Research Institution
      Tottori University
  •  Elucidation of the process by which social skills develop using noninvasive functional neuroimaging to quantitate multiple individual interactions and analysze their interaction

    • Principal Investigator
      SADATO Norihiro
    • Project Period (FY)
      2005 – 2009
    • Research Category
      Grant-in-Aid for Scientific Research (S)
    • Research Field
      Cognitive science
    • Research Institution
      National Institute for Physiological Sciences
  •  ニーマンピック病C型の神経変性機構の解明

    • Principal Investigator
      二宮 治明
    • Project Period (FY)
      2004
    • Research Category
      Grant-in-Aid for Scientific Research on Priority Areas
    • Review Section
      Biological Sciences
    • Research Institution
      Tottori University
  •  Establishment of new therapeutic strategies for neurogenetic disorders during childhoodPrincipal Investigator

    • Principal Investigator
      OHNO Kousaku
    • Project Period (FY)
      2004 – 2006
    • Research Category
      Grant-in-Aid for Scientific Research (B)
    • Research Field
      Pediatrics
    • Research Institution
      Tottori University
  •  ニーマン・ピック病C型の神経変性に対する実験的治療Principal Investigator

    • Principal Investigator
      大野 耕策
    • Project Period (FY)
      2003
    • Research Category
      Grant-in-Aid for Scientific Research on Priority Areas
    • Review Section
      Biological Sciences
    • Research Institution
      Tottori University
  •  Analysis of the relationship between Amyloid β protein and cholesterol in Alzheimer's disease.

    • Principal Investigator
      URAKAMI Katsuya
    • Project Period (FY)
      2003 – 2005
    • Research Category
      Grant-in-Aid for Scientific Research (C)
    • Research Field
      Neurology
    • Research Institution
      National University Corporation Tottori University
  •  ニーマン・ピック病C型の神経変性機構の解明Principal Investigator

    • Principal Investigator
      大野 耕策
    • Project Period (FY)
      2002
    • Research Category
      Grant-in-Aid for Scientific Research on Priority Areas
    • Review Section
      Biological Sciences
    • Research Institution
      Tottori University
  •  Development of a new molecular therapeutic drug for brain pathology in lysosomal storage diseases

    • Principal Investigator
      SUZUKI Yoshiyuki
    • Project Period (FY)
      2002 – 2004
    • Research Category
      Grant-in-Aid for Scientific Research (A)
    • Research Field
      応用薬理学・医療系薬学
    • Research Institution
      International University of Health and Welfare
  •  ニーマン・ピック病C型の神経変性機構の解明Principal Investigator

    • Principal Investigator
      大野 耕策
    • Project Period (FY)
      2001
    • Research Category
      Grant-in-Aid for Scientific Research on Priority Areas (C)
    • Review Section
      Biological Sciences
    • Research Institution
      Tottori University
  •  ニーマン・ピック病C型の神経変性機構の解明Principal Investigator

    • Principal Investigator
      大野 耕策
    • Project Period (FY)
      2000
    • Research Category
      Grant-in-Aid for Scientific Research on Priority Areas (C)
    • Review Section
      Biological Sciences
    • Research Institution
      Tottori University
  •  ヒト血漿リポ蛋白ならびに赤血球の氷温保存法の臨床的応用

    • Principal Investigator
      猪川 嗣朗
    • Project Period (FY)
      1998 – 2000
    • Research Category
      Grant-in-Aid for Exploratory Research
    • Research Field
      Laboratory medicine
    • Research Institution
      Tottori University
  •  PATHOPHYSIOLOGY OF CARBOHYDRATE-DEFICIET GLYCOPROTEIN SYNDROMEPrincipal Investigator

    • Principal Investigator
      OHNO Kousaku
    • Project Period (FY)
      1998 – 1999
    • Research Category
      Grant-in-Aid for Scientific Research (C)
    • Research Field
      Pediatrics
    • Research Institution
      TOTTORI UNIVERSITY
  •  Research on the basic defect of carbohydrate-deficient glycoprotein syndromePrincipal Investigator

    • Principal Investigator
      OHNO Kousaku
    • Project Period (FY)
      1996 – 1997
    • Research Category
      Grant-in-Aid for Scientific Research (C)
    • Research Field
      Pediatrics
    • Research Institution
      Tottori University
  •  遺伝子治療の基礎的研究:小児神経疾患のモデル動物を用いた神経細胞移植Principal Investigator

    • Principal Investigator
      大野 耕策
    • Project Period (FY)
      1995
    • Research Category
      Grant-in-Aid for General Scientific Research (C)
    • Research Field
      Pediatrics
    • Research Institution
      Tottori University
  •  Etiology and Pathogenesis of CDG Syndrome

    • Principal Investigator
      SUZUKI Yoshiyuki
    • Project Period (FY)
      1994 – 1995
    • Research Category
      Grant-in-Aid for General Scientific Research (B)
    • Research Field
      Pediatrics
    • Research Institution
      The Tokyo Metropolitan Institute of Medical Science
  •  GENETIC STUDY OF A CHILDHOOD DISEASE AFFECTNG INTRACELLULAR CHOLESTEROL TRANSPORTPrincipal Investigator

    • Principal Investigator
      OHNO Kousaku
    • Project Period (FY)
      1992 – 1993
    • Research Category
      Grant-in-Aid for General Scientific Research (C)
    • Research Field
      Pediatrics
    • Research Institution
      TOTTORI UNIVERSITY
  •  Experimental study on developmental delay and promotion of the brain.

    • Principal Investigator
      ANDO Yukinori, TAKASHIMA Sachio
    • Project Period (FY)
      1986 – 1987
    • Research Category
      Grant-in-Aid for General Scientific Research (C)
    • Research Field
      Pediatrics
    • Research Institution
      Division of Child Neurology, Tottori University School of Medicine

All 2011 2010 2009 2008 2007 2006 2004 2003 2002

All Journal Article Presentation Book Patent

  • [Book] 今日の小児治療指針 第14版「Niemann-Pick病」(大関武彦, 古川漸, 横田俊一郎 編)2006

    • Author(s)
      大野耕策
    • Total Pages
      840
    • Publisher
      医学書院
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Book] 今日の小児治療指針 第14版「Niemann-Pick病」(大関武彦、古川漸、横田俊一郎 編)2006

    • Author(s)
      大野耕策
    • Total Pages
      840
    • Publisher
      医学書院
    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Book] Niemann-Pick diseases. In "Today's Therapy for Diseases of Children", 14^<th> edition. (Ohzeki T, Furukawa H, Yokota S (eds))2006

    • Author(s)
      Kousaku Ohno
    • Total Pages
      840
    • Publisher
      Igakushoin
    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Chemical chaperone therapy : chaperone effect on mutant enzyme and cellular pathophysiology in β-galactosidase deficiency.2011

    • Author(s)
      Higaki K, Li L, Udin Bahrudin, Okuzawa S, Takamura A, Yamamoto K, Adachi K, Rubigilda C. Paraguison, Takai T, Ikehata H, Tominaga L, Hisatome I, Iida M, Ogawa S, Matsuda J, Ninomiya H, Sakakibara Y, Ohno K, Suzuki Y, Nanba E.
    • Journal Title

      Human Mutation. in press

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] Chemical chaperone therapy : Luciferase assay for screening of b-galactosidase mutations.2010

    • Author(s)
      Li L, Higaki K, Ninomiya H, Luan Z, Iida M, Ogawa S, Suzuki Y, Nanba E, Ohno K.
    • Journal Title

      Mol Genet Metab 101(4)

      Pages: 364-369

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] ライソゾーム病の治療シャペロン療法2010

    • Author(s)
      大野耕策
    • Journal Title

      血液フロンティア 20(4)

      Pages: 69-77

    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] The pharmacological chaperone effect of N-octyl-beta-valienamine on human mutant acid beta-glucosidases.2010

    • Author(s)
      Luan Z, Li L, Ninomiya H, Ohno K, Ogawa S, Kubo T, Iida M, Suzuki Y.
    • Journal Title

      Blood Cells Mot Dis. 44(1)

      Pages: 48-54

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] A fluorescent SP2-iminosugar with pharmacological chaperone activity for Gaucher disease : Cellular uptake and intracellular distribution studies.2010

    • Author(s)
      Luan Z, Higaki K, Aguilar-Moncayo M, Li L, Ninomiya H, Nanba E, Ohno K, Garcia-Moreno M, Mellet CO, Fernandez JMG, Suzuki Y.
    • Journal Title

      ChemBioChem 11(17)

      Pages: 2453-2464

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] ライソゾーム病の治療 シャペロン療法2010

    • Author(s)
      大野耕策
    • Journal Title

      血液フロンティア 20

      Pages: 69-77

    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] The effect of N-octyl-β-valienamine on β-glucosidase activity in tissues of normal mice.2010

    • Author(s)
      Luan Z, Ninomiya H, Ohno K, Ogawa S, Kubo T, Iida M, Suzuki Y.
    • Journal Title

      Brain Dev 32(10)

      Pages: 805-809

    • NAID

      10029559648

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] Chaperone activity of bicyclic nojirimycin analogues for Gaucher mutations in comparison with N-(n-nonyl)deoxynojirimycin.2009

    • Author(s)
      Luan Z, Higaki K, Aguilar-Moncayo M, Ninomiya H, Ohno K, Garcia-Moreno MI, Ortiz Mellet C, Garcia Fernandez JM, Suzuki Y.
    • Journal Title

      Chembiochem. 10(17)

      Pages: 2780-2792

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] Enhanced autophagy and mitochondrial aberrations in murine G(M1)-gangliosidosis.2008

    • Author(s)
      Takamura A, Bigaki K, Kajimaki K, Otsuka S, Ninomiya H, Natsuda J, Ohno K, Suzuki Y, Nanba E.
    • Journal Title

      Biochem Biophys Res Commun 367

      Pages: 616-622

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] Brainstem neuropathology in a mouse model of Niemann-Pick disease type C.2008

    • Author(s)
      Luan Z, Saito Y, Miyata H, Ohama E, Ninomiya H, Ohno K.
    • Journal Title

      J Neurol Sci 268

      Pages: 108-116

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] Brainstem neuropathology in a mouse model of Niemann-Pick disease type C.2008

    • Author(s)
      Luan Z, Saito Y, Miyata H, Ohama E, Ninomiya H, Ohno K.
    • Journal Title

      J Neurol Sci. 268(1-2)

      Pages: 108-116

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] Enhanced autophagy and mitochondrial aberrations in murine G(M1)-gangliosidosis.2008

    • Author(s)
      Takamura A, Higaki K, Kajimaki K, Otsuka S, Ninomiya H, Matsuda J, Ohno K, Suzuki Y, Nanba E.
    • Journal Title

      Biochem Biophys Res Commun. 367(3)

      Pages: 616-622

    • Peer Reviewed
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Journal Article] Endosomal accumulation of Toll-like receptor 4 causes constitutive secretion of cytokines and activation of STATs in Niemann-Pick disease type C fibroblasts : a potential basis for glial cell activation in the NPC brain.2007

    • Author(s)
      Suzuki M, Sugimoto Y, Ohsaki Y, Ueno M, Kato S, Kitamura Y, Hosokawa H, Davies JP, Ioannou YA, Vanier MT, Ohno K, Ninomiya H.
    • Journal Title

      J Neurosci 27

      Pages: 1879-1891

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Enzyme enhancement activity of N-octyl-beta-valienamine on beta-glucosidase mutants associated with Gaucher disease.2007

    • Author(s)
      Lei K, Ninomiya H, Suzuki M, Inoue T, Sawa M, Iida M, Ida H, Eto Y, Ogawa S, Ohno K, Suzuki Y
    • Journal Title

      Biochim Biophys Acta 1772

      Pages: 587-596

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Enzyme enhancement activity of N-octyl-beta-valienamine on beta-glucosidase mutants associated with Gaucher disease.2007

    • Author(s)
      Lei K, Ninomiya H, Suzuki M, Inoue T, Sawa M, Iida M, Ida H, Eto Y, Ogawa S, Ohno K, Suzuki Y.
    • Journal Title

      Biochim Biophys Acta. 1772

      Pages: 587-96

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Endosomal accumulation of Toll-like receptor 4 causes constitutive secretion of cytokines and activation of STATs in Niemann-Pick disease type C fibroblasts : a potential basis for glial cell activation in the NPC brain. 27 : 1879-1891,20072007

    • Author(s)
      Suzuki M, Sugimoto Y, Ohsaki Y, Ueno M, Kato S, Kitamura Y, Hosokawa H, Davies JP, Ioannou YA, Vanier MT, Ohno K, Ninomiya H
    • Journal Title

      Journal of Neuroscience 27

      Pages: 1879-1891

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Chemical synthesis of the 3-sulfooxy-7-N-acetylglucosaminyl-24- amidated conjugates of 3β,7β-dihydroxy-5-cholen-24-oic acid, and related compounds : Unusual, major metabolites of bile acid in a patient with Niemann-Pick disease type C1.2006

    • Author(s)
      Iida T, Kakiyama G, Hibiya Y, Miyata S, Inoue T, Ohno K, Goto T, Mano N, Goto J, Nambara T, A F Hofmann
    • Journal Title

      Steroids 71

      Pages: 18-29

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Neural substrates of sarcasm : a functional magnetic-resonance imaging study.2006

    • Author(s)
      Uchiyama H, Seki A, Kageyama H, Saito DN, Koeda T, Ohno K, Sadato N
    • Journal Title

      Brain Res 1124

      Pages: 100-110

    • Data Source
      KAKENHI-PROJECT-17100003
  • [Journal Article] Cholesterol depletion facilitates ubiquitylation of NPC1 and its association with SKD1/Vps4.2006

    • Author(s)
      Ohsaki Y, Sugimoto Y, Suzuki M, Hosokawa H, Yoshimori T, Davies JP, Ioannou YA, Vanier MT, Ohno K, Ninomiya H
    • Journal Title

      J Cell Sci 119

      Pages: 2643-2653

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Activity of N-alkyl-β-valienamine sa a Chemical Chaperone for β-glucosidase Mutants Associated with Gaucher Disease.2006

    • Author(s)
      K Ohno, et al.
    • Journal Title

      10~<th> International Child Neurology Congress

      Pages: 135-138

    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Cholesterol depletion facilitates ubiquitylation of NPC1 and its association with SKD1/Vps4.2006

    • Author(s)
      Ohsaki Y, Sugimoto Y, Suzuki M, Hosokawa H, Yoshimori T, Davies JP, Ioannou YA, Vanier MT, Ohno K, Ninomiya H.
    • Journal Title

      J Cell Sci. 119

      Pages: 2643-2653

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Chemical synthesis of the 3-sulfooxy-7-N-acetylglucosaminyl-24-amidated conjugates of 3β,7β-dihydroxy-5-cholen-24-oic acid, and related compounds : Unusual, major metabolites of bile acid in a patient with Niemann-Pick disease type C1.2006

    • Author(s)
      Iida T, Kakiyama G, Hibiya Y, Miyata S, Inoue T, Ohno K, Goto T, Mano N, Goto J, Nambara T, A F Hofmann.
    • Journal Title

      Steroids 71

      Pages: 18-29

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Axonal dystrophy of dorsal root ganglion sensory neurons in a mouse model of Niemann-Pick disease type C.Experimental2004

    • Author(s)
      Ohara S, Ukita Y, Ninomiya H, Ohno K
    • Journal Title

      Experimental Neurology 187

      Pages: 289-298

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] N-Octyl-β-valienamine up-regulates activity of F213I mutant β-glucosidase in cultured cells : a potential chemical chaperone therapy for Gaucher disease2004

    • Author(s)
      Lin H, Sugimoto Y, Ohsaki Y, Ninomiya H, Oka A, Taniguchi M, Ida H, Eto Y, Ogawa S, Matsuzaki Y, Sawa M, Inoue T, Higaki K, Nanba E, Ohno K, Suzuki Y
    • Journal Title

      Biochim Biophys Acta 1689

      Pages: 219-228

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-14207106
  • [Journal Article] N-Octyl-β-valienamine up-regulates activity of F213I mutant β-glucosidase in cultured cells : a potential chemical chaperone therapy for Gaucher disease.2004

    • Author(s)
      Lin H, Sugimoto Y, Ohsaki Y, Ninomiya H, Oka A, Taniguchi M, Ida H, Eto Y, Ogawa S, Matsuzaki Y, Sawa M, Inoue T, Higaki K, Nanba E, Ohno K, Suzuki Y.
    • Journal Title

      Biochim Biophys Acta. 1689

      Pages: 219-228

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Degeneration of cholecystokinin-immunoreactive afferents to the VPL thalamus in a mouse model of Niemann-Pick disease type C2004

    • Author(s)
      Ohara S, Ukita Y, Ninomiya H, Ohno K
    • Journal Title

      Brain Research 1022

      Pages: 244-246

    • Data Source
      KAKENHI-PROJECT-14207106
  • [Journal Article] Increased NPC1 mRNA in skin fibroblasts from Niemann-Pick disease type C patients.2004

    • Author(s)
      Yamamoto T, Feng J-H, Higaki K, Taniguchi M, Nanba E, Ninomiya H, Ohno K.
    • Journal Title

      Brain Dev. 26

      Pages: 245-250

    • NAID

      10020552645

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Axonal dystrophy of dorsal root ganglion sensory neurons in a mouse model of Niemann-Pick disease type C.2004

    • Author(s)
      Ohara S, Ukita Y, Ninomiya H, Ohno K.
    • Journal Title

      Experimental Neurology. 187

      Pages: 289-298

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Novel TSC2 mutations and decreased expression of tuberin in cultured tumor cells with an insertion mutation2004

    • Author(s)
      Feng J-H, Yamamoto T, Nanba E, Ninomiya H. Oka A. Ohno K
    • Journal Title

      HUMAN MUTATION #696(Online)

    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Increased NPC1 mRNA in skin fibroblasts from Niemann-Pick disease type C patients2004

    • Author(s)
      Yamamoto T, Feng J-H, Higaki K, Taniguchi M, Nanba E. Ninomiya H, Ohno K
    • Journal Title

      Brain Dev 26

      Pages: 245-250

    • NAID

      10020552645

    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Degeneration of cholecystokinin-immunoreactive afferents to the VPL thalamus in a mouse model of Niemann-Pick disease type C.2004

    • Author(s)
      Ohara S, Ukita Y, Ninomiya H, Ohno K.
    • Journal Title

      Brain Research 1022

      Pages: 244-246

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Reduced sensitivity of Niemann-Pick C1-deficient cells to θ-toxin (perfringolysin O) : sequestration of toxin to raft-enriched membrane vesicles.2004

    • Author(s)
      Ohsaki Y, Sugimoto Y, Kaidoh T, Shimada Y, Ohno-Iwashita Y, Joanna P. Davies, Yiannis A. Ioannou, Ohno K, Ninomiya H
    • Journal Title

      Histochem Cell Biol 121

      Pages: 263-272

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Degeneration of cholecystokinin-immunoreactive afferents to the VPL thalamus in mouse model of Niemann-Pick disease type C2004

    • Author(s)
      Ohara S, Ukita Y, Ninomiya H, Ohno K
    • Journal Title

      Brain Research 1022

      Pages: 244-246

    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Axonal dystrophy of dorsal root ganglion sensory neurons in a mouse model of Niemann-Pick disease type C2004

    • Author(s)
      Ohara S, Ukita Y, Ninomiya H, Ohno K
    • Journal Title

      Experimental Neurology 187

      Pages: 289-298

    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] N-Octyl-β-valienamine up-regulates activity of F213I mutant β-glucosidase in cultured cells : a potential chemical chaperone therapy for Gaucher disease.2004

    • Author(s)
      Lin H, Sugimoto Y, Ohsaki Y, Ninomiya H, Oka A, Taniguchi M, Ida H, Eto Y, Ogawa S, Matsuzaki Y, Sawa M, Inoue T, Higaki K, Nanba E, Ohno K, Suzuki Y
    • Journal Title

      Biochim Biophys Acta 1689

      Pages: 219-228

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Degeneration of cholecystokinin- immunoreactive afferents to the VPL thalamus in a mouse model of Niemann-Pick disease type C.2004

    • Author(s)
      Ohara S, Ukita Y, Ninomiya H, Ohno K
    • Journal Title

      Brain Research 1022

      Pages: 244-246

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Reduced sensitivity of Niemann-Pick C1-deficient cells to θ-toxin (perfringolysin O) : sequestration of toxin to raft-enriched membrane vesicles.2004

    • Author(s)
      Ohsaki Y, Sugimoto Y, Kaidoh T, Shimada Y, Ohno-Iwashita Y, Joanna P.Davies, Yiannis A.Ioannou, Ohno K, Ninomiya H.
    • Journal Title

      Histochem Cell Biol. 121

      Pages: 263-272

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] N-Octyl-β-valienamine up-regulates activity of F213I mutant β-glucosidase in cultured cells : a potential chemical chaperone therapy for Gaucher disease2004

    • Author(s)
      Lin H, Sugimoto Y, Ohsaki Y, Ninomiya H, Oka A, Taniguchi M, Ida H, Eto Y, Ogawa S, Matsuzaki Y, Sawa M, Inoue T, Higaki K, Nanba E, Ohno K, Suzuki Y
    • Journal Title

      Biochem Biophys Acta 1689

      Pages: 219-228

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-14207106
  • [Journal Article] Increased NPC1 mRNA in skin fibroblasts from Niemann-Pick disease type C patients.2004

    • Author(s)
      Yamamoto T, Feng J-H, Higaki K, Taniguchi M, Nanba E, Ninomiya H, Ohno K
    • Journal Title

      Brain Dev 26

      Pages: 245-250

    • NAID

      10020552645

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-16390302
  • [Journal Article] Chemical chaperone therapy for brain pathology in G_<M1>-gangliosidosis2003

    • Author(s)
      Matsuda J, Suzuki O, Oshima A, 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
    • Journal Title

      Proc Natl Acad Sci USA 100

      Pages: 15912-15917

    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-14207106
  • [Journal Article] Antisense in vivo knockdown of synaptotagmin I and synapsin I by HVJ-liposome mediated gene transfer modulates ischemic injury of hippocampus in opposing ways2003

    • Author(s)
      Iwakuma M, Anzai T, Kobayashi S, Ogata M, Kaneda Y, Ohno K, Saji M
    • Journal Title

      Neurosci Res 45

      Pages: 285-296

    • NAID

      50000792044

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-14207106
  • [Journal Article] Chemical chaperone therapy for brain pathology in G_<M1>-gangliosidosis2003

    • Author(s)
      Matsuda J, Suzuki O, Oshima A, 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
    • Journal Title

      Proc Natl Acad Sci USA 100

      Pages: 15912-15917

    • Description
      「研究成果報告書概要(欧文)」より
    • Data Source
      KAKENHI-PROJECT-14207106
  • [Patent] Carba-sugar amine derivativeand glycolipid metabolic disorder treating agent containing the same as active agent2010

    • Inventor(s)
      Ogawa S, SuzukiY, Nanba E, MatsudaJm Ohno K
    • Industrial Property Rights Holder
      生化学工業
    • Acquisition Date
      2010-05-25
    • Overseas
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Patent] Compuestos potenciadores de la actividad de glicosidasas mutantes2009

    • Inventor(s)
      Jose Manuel Garcia Fernandez, Carmen Ortiz Mellet, Yoshiyuki Suzuki, Kousaku Ohno
    • Industrial Property Rights Holder
      鳥取大学、国際医療福祉大学、スペイン・セビリア大学
    • Acquisition Date
      2009-11-05
    • Overseas
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Patent] Carba-sugar amine derivatives and treatments for disorder of glycolipid metabolism containing the same as the active ingredient2002

    • Inventor(s)
      小川 誠一郎, 鈴木 義之, 難波 栄二, 松田 潤一郎, 大野 耕策
    • Industrial Property Rights Holder
      生化学工業株式会社
    • Filing Date
      2002-09-02
    • Description
      「研究成果報告書概要(和文)」より
    • Data Source
      KAKENHI-PROJECT-14207106
  • [Presentation] 遺伝性小児神経疾患の治療について2011

    • Author(s)
      大野耕策
    • Organizer
      第1回由布院・児湯小児神経学研究会
    • Place of Presentation
      大分
    • Year and Date
      2011-03-18
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Presentation] 神経変性疾患と先天代謝異常症2010

    • Author(s)
      大野耕策
    • Organizer
      第52回日本先天代謝異常学会
    • Place of Presentation
      大阪
    • Year and Date
      2010-10-23
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Presentation] Molecular pathology : Gaucherdisease2010

    • Author(s)
      大野耕策
    • Organizer
      シャペロン療法国際シンポジウム
    • Place of Presentation
      米子
    • Year and Date
      2010-07-29
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Presentation] ライソゾーム病の新しい低分子治療薬の開発2010

    • Author(s)
      大野耕策
    • Organizer
      第15回日本ライソゾーム病研究会
    • Place of Presentation
      東京
    • Year and Date
      2010-12-11
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Presentation] Chemical chaperone for Gaucher disease2010

    • Author(s)
      大野耕策
    • Organizer
      第1回アジア先天代謝異常学会議(The 1st ACIMD)
    • Place of Presentation
      福岡
    • Year and Date
      2010-03-09
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Presentation] 医師とともに治療する薬剤業務-遺伝性ライソゾーム病に対する新しい治療と薬剤部との連携-2010

    • Author(s)
      大野耕策
    • Organizer
      第49回日本薬学会・日本薬剤師会・日本病院薬剤師会中国四国支部学術大会
    • Place of Presentation
      米子
    • Year and Date
      2010-11-06
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Presentation] 遺伝性神経疾患の神経変性機構の解明と治療法の開発2009

    • Author(s)
      大野耕策
    • Organizer
      第51回日本小児神経学会総会 会長講演
    • Place of Presentation
      米子
    • Year and Date
      2009-05-28
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Presentation] リソゾーム病の新しい治療を中心に2009

    • Author(s)
      大野耕策
    • Organizer
      第4回成育代謝異常症研究会
    • Place of Presentation
      東京
    • Year and Date
      2009-12-04
    • Data Source
      KAKENHI-PROJECT-20390297
  • [Presentation] ゴーシェ病へのケミカルシャペロン療法2009

    • Author(s)
      大野耕策
    • Organizer
      第1回カプアセミナー
    • Place of Presentation
      千葉
    • Year and Date
      2009-08-22
    • Data Source
      KAKENHI-PROJECT-20390297
  • 1.  NINOMIYA Haruaki (80212124)
    # of Collaborated Projects: 7 results
    # of Collaborated Products: 20 results
  • 2.  佐治 真理 (50114179)
    # of Collaborated Projects: 2 results
    # of Collaborated Products: 0 results
  • 3.  INOUE Takehiko (80346361)
    # of Collaborated Projects: 2 results
    # of Collaborated Products: 6 results
  • 4.  NANBA Eiji (40237631)
    # of Collaborated Projects: 2 results
    # of Collaborated Products: 4 results
  • 5.  SUZUKI Yoshiyuki (90010389)
    # of Collaborated Projects: 2 results
    # of Collaborated Products: 4 results
  • 6.  HIGAKI Katsumi (90294321)
    # of Collaborated Projects: 2 results
    # of Collaborated Products: 0 results
  • 7.  ANDO Yukinori (90168047)
    # of Collaborated Projects: 2 results
    # of Collaborated Products: 0 results
  • 8.  TAKASHIMA Sachio (70038743)
    # of Collaborated Projects: 2 results
    # of Collaborated Products: 0 results
  • 9.  岡 明 (00251273)
    # of Collaborated Projects: 2 results
    # of Collaborated Products: 0 results
  • 10.  SADATO Norihiro (00273003)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 1 results
  • 11.  MAYUMI Mitsuhumi (70135581)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 12.  NAKAI Akio (50240784)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 13.  MATSUKI Ken-ichi (10157282)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 14.  KOEDA Tatsuya (70225390)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 1 results
  • 15.  ITAKURA Shoji (50211735)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 16.  OZAKI Toru (00000208)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 17.  YONEKURA Yoshiharu (60135572)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 18.  SUGIURA Titose (90325027)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 19.  AKABOSHI Shinjiro (90231810)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 20.  OSHIMA Akihiro (20203763)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 21.  ITOH Kohji (00184656)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 22.  SAKURABA Hitoshi (60114493)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 23.  KUROSAWA Mieko (30178131)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 24.  MATSUDA Junichiro (60181731)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 2 results
  • 25.  URAKAMI Katsuya (30213507)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 26.  TANIGUCHI Miyako (50335527)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 27.  NAKASHIMA Kenji (70144673)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 28.  WAKUTANI Yousuke (10322215)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 29.  MITO Takashi (00166068)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 30.  石田 玄 (30252860)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 31.  猪川 嗣朗 (70032183)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 32.  井上 貴夫 (20116312)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 33.  下村 登規夫 (00216136)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 34.  西川 健一 (90144675)
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 35.  IIDA Masami
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results
  • 36.  松崎 祐二
    # of Collaborated Projects: 1 results
    # of Collaborated Products: 0 results

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