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Clarify the abnormal protein causing ER stress in ALS and therapeutic approach

Research Project

Project/Area Number 19590997
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionKyushu University

Principal Investigator

KIKUCHI Hitoshi  Kyushu University, 大学病院, 特任講師 (60322765)

Co-Investigator(Kenkyū-buntansha) TATEISHI Takahisa  九州大学, 大学病院, 助教 (50423546)
KAWAMURA Nobutoshi  九州大学, 大学病院, 非常勤助教 (00432930)
Project Period (FY) 2007 – 2008
Project Status Completed (Fiscal Year 2008)
Budget Amount *help
¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2008: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2007: ¥2,860,000 (Direct Cost: ¥2,200,000、Indirect Cost: ¥660,000)
Keywords神経病態生化学 / 筋萎縮性側索硬化症 / 小胞体ストレス / プロテアーゼ / ALS / 異常蛋白質
Research Abstract

筋萎縮性側索硬化症(ALS)は運動ニューロンの選択的変性に加え, 運動ニューロン胞体内での神経細胞内封入体の出現を特徴とし, 蛋白分解系の異常ならびに細胞骨格異常による神経細胞死が注目されている. 本研究より, ALSでは, 小胞体内で蓄積した異常変性蛋白が細胞死を誘導し, 封入体を形成することが考えられた. さらにALSではカルパインの活性化が病態形成に関与し, カテプシンBが運動ニューロン死に対して神経防御的に作用していることが考えられた.

Report

(3 results)
  • 2008 Annual Research Report   Final Research Report ( PDF )
  • 2007 Annual Research Report
  • Research Products

    (24 results)

All 2009 2008 2007

All Journal Article (16 results) (of which Peer Reviewed: 9 results) Presentation (8 results)

  • [Journal Article] Cortical kindling in a focal freeze lesion rat model2009

    • Author(s)
      Takase K, Shigeto H, Suzuki SO, Kikuchi H, Ohyagi Y, Kira J.
    • Journal Title

      J Clin Neurosci 16

      Pages: 94-98

    • Related Report
      2008 Final Research Report
  • [Journal Article] Cortical kindling in a focal freeze lesion rat model.2009

    • Author(s)
      Takase K, Shigeto H, Suzuki so, Kikuchi H, Ohyagi Y, Kira J.
    • Journal Title

      J Clin Neurosci 16

      Pages: 94-98

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Anterior periventricular linear lesions in optic-spinal multiple sclerosis : a combined neuroimaging and neuropathological study2008

    • Author(s)
      Matsushita T, Matsuoka T, Ishizu T, Kikuchi H, 他5名
    • Journal Title

      Multiple Sclerosis 14

      Pages: 343-353

    • Related Report
      2008 Final Research Report
  • [Journal Article] Prenatal freeze lesioning produces epileptogenic focal cortical dysplasia2008

    • Author(s)
      Takase K, Shigeto H, Suzuki SO, Kikuchi H, Ohyagi Y, Kira J.
    • Journal Title

      Epilepsia 49

      Pages: 997-1010

    • Related Report
      2008 Final Research Report
  • [Journal Article] Anterior periventricular linear lesions in optic-spinal multiplesclerosis : a combined neuroimaging and neuropathological study.2008

    • Author(s)
      Matsushita T, Matsuoka T, Ishizu T, Kikuchi H, 他5名
    • Journal Title

      Multiple Sclerosis 14

      Pages: 343-353

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Prenatal freeze lesioning produces epileptogenic focal cortical dysplasia.2008

    • Author(s)
      Takase K, Shigeto H, Suzuki so, Kikuchi H, Ohyagi Y, Kira J.
    • Journal Title

      Epilepsia. 49

      Pages: 997-1010

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Anterior periventricular linear lesions in optic-spinal multiple sclerosis:a combined neuroimaging and neuropathological study2008

    • Author(s)
      Matsushita T, Matsuoka T, Ishizu T, Kikuchi H, 他5名
    • Journal Title

      Multiple Sclerosis (印刷中)(in press)

    • Related Report
      2007 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Prenatal freeze lesioning produces epileptogenic focal cortical dysplasia2008

    • Author(s)
      Takase KI, Shigeto H, Suzuki SO, Kikuchi H, Ohyagi Y, Kira JI
    • Journal Title

      Epilepsia (印刷中)(in press)

    • Related Report
      2007 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Increased IL-13-producing T cells in ALS : positive correlations with disease severity and progression rate2007

    • Author(s)
      Shi N, Kawano Y, Tateishi T, Kikuchi H, 他3名
    • Journal Title

      J Neuroimmunol 182

      Pages: 232-235

    • Related Report
      2008 Final Research Report
  • [Journal Article] Intraneuronal amyloid beta42 enhanced by heating but counteracted by formic acid2007

    • Author(s)
      Ohyagi Y, Tsuruta Y, Motomura K, Miyoshi K, Kikuchi H, 他3名
    • Journal Title

      J Neurosci Methods

      Pages: 134-138

    • Related Report
      2008 Final Research Report
  • [Journal Article] Heterogeneity of aquaporin-4 autoimmunity and spinal cord lesions in multiple sclerosis in Japanese2007

    • Author(s)
      Matsuoka T, Matsushita T, Kawano Y, Osoegawa M, Ochi H, Ishizu T, Minohara. M, Kikuchi H, 他3名
    • Journal Title

      Brain 130

      Pages: 1206-1223

    • Related Report
      2008 Final Research Report
  • [Journal Article] Slowly progressive distal muscular atrophy of the bilateral upper limbs (O'Sullivan-McLeod syndrome) partially alleviated by intravenous immunoglobulin therapy2007

    • Author(s)
      Kawano Y, Nagara Y, Murai H, Kikuchi H, Ohyagi Y, Kira J.
    • Journal Title

      Intern Med 46

      Pages: 515-518

    • NAID

      130000078746

    • Related Report
      2008 Final Research Report
  • [Journal Article] Increased IL-13-producing T cells in ALS:positive correlations with disease severity and progression rate2007

    • Author(s)
      Shi N, Kawano Y, Tateishi T, Kikuchi H, 他3名
    • Journal Title

      J Neuroimmunol 182

      Pages: 232-235

    • Related Report
      2007 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Intraneuronal amyloid beta42 enhanced by heating but counteracted by formicacid2007

    • Author(s)
      Ohyagi Y, Tsuruta Y, Motomura K, Miyoshi K, Kikuchi H, 他3名
    • Journal Title

      J Neurosci Methods 159

      Pages: 134-138

    • Related Report
      2007 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Heterogeneity of aquaporin-4 autoimmunity and spinal cord lesions in multiple-sclerosis in Japanese2007

    • Author(s)
      Matsuoka T, Matsushita T, Kawano Y, Osoegawa M, Ochi H, Ishizu T, Minohara M, Kikuchi H, 他3名
    • Journal Title

      Brain 130

      Pages: 1206-1223

    • Related Report
      2007 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Slorvly progressive distal muscular atrophy of the bilateral upper limbs(O'Sullivan-McLeod syndrome) partially alleviated by intravenous immunoglobulin therapy2007

    • Author(s)
      Kawano Y, Nagara Y, Murai H, Kikuchi H, Ohyagi Y, Kira J.
    • Journal Title

      Intern Med 46

      Pages: 515-518

    • Related Report
      2007 Annual Research Report
    • Peer Reviewed
  • [Presentation] 変異SOD1トランスジェニックマウスにおけるミクログリア機能障害2009

    • Author(s)
      山崎亮, 福永真実, 立石貴久, 田中正人, 菊池仁志, 大八木保政, 吉良潤一
    • Organizer
      第21回日本神経免疫学会学術集会
    • Place of Presentation
      大阪
    • Related Report
      2008 Final Research Report
  • [Presentation] 変異SODIトランスジェニツクマウスにおけるミクロクリア機能障害2009

    • Author(s)
      山崎 亮, 福永真実, 立石貴久, 田中 正人, 菊池 仁志, 大八木保政, 吉良 潤一.
    • Organizer
      第21回日本神経免疫学会学術集会
    • Place of Presentation
      大阪
    • Related Report
      2008 Annual Research Report
  • [Presentation] mSODl G93Aマウスにおける、VEGFとHIF-lαの発現の解析、2008

    • Author(s)
      立石貴久, 田中正人, 山崎亮, 菊池仁志, 吉良潤一
    • Organizer
      日本神経学会総会
    • Place of Presentation
      横浜
    • Year and Date
      2008-05-16
    • Related Report
      2008 Annual Research Report
  • [Presentation] ALS患者におけるNIPPV下での経皮内視鏡的胃ろう造設術の検討2008

    • Author(s)
      菊池仁志, 田代博文, 大久保正一, 久米徹
    • Organizer
      第49回日本神経学会総会
    • Place of Presentation
      横浜
    • Year and Date
      2008-05-16
    • Related Report
      2008 Annual Research Report
  • [Presentation] 変異SODl-Tgマウスにおける神経細胞脆弱性とミクロクリア機能障害、2008

    • Author(s)
      山崎 亮, 田中正人, 立石貴久, 菊池仁志, 吉良潤一
    • Organizer
      日本神経学会総会
    • Place of Presentation
      横浜
    • Year and Date
      2008-05-15
    • Related Report
      2008 Annual Research Report
  • [Presentation] 変異SOD1-Tgマウスにおける神経細胞脆弱性とミクログリア機能障害2008

    • Author(s)
      山崎亮, 田中正人, 立石貴久, 菊池仁志, 吉良潤一
    • Organizer
      日本神経学会総会
    • Place of Presentation
      横浜
    • Related Report
      2008 Final Research Report
  • [Presentation] mSOD1 G93Aマウスにおける, VEGFとHIF-1αの発現の解析2008

    • Author(s)
      立石貴久, 田中正人, 山崎亮, 菊池仁志, 吉良潤一
    • Organizer
      日本神経学会総会
    • Place of Presentation
      横浜
    • Related Report
      2008 Final Research Report
  • [Presentation] ALS患者におけるNIPPV下での経皮内視鏡的胃ろう造設術の検討2008

    • Author(s)
      菊池仁志, 田代博文, 大久保正一, 久米徹
    • Organizer
      日本神経学会総会
    • Place of Presentation
      横浜
    • Related Report
      2008 Final Research Report

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Published: 2007-04-01   Modified: 2016-04-21  

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