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The characterization of TDP-43 aggregation in Amyotrophic Lateral Sclerosis (ALS)

Research Project

Project/Area Number 21790850
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeSingle-year Grants
Research Field Neurology
Research InstitutionKeio University

Principal Investigator

NISHIMOTO Yoshinori  Keio University, 医学部, 助教 (30398622)

Project Period (FY) 2009 – 2010
Project Status Completed (Fiscal Year 2010)
Budget Amount *help
¥2,730,000 (Direct Cost: ¥2,100,000、Indirect Cost: ¥630,000)
Fiscal Year 2010: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2009: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
KeywordsALS(筋萎縮性側索硬化症) / TDP-43 / caspase-3 / aggresome / stress granule / RNA / 筋萎縮性側索硬化症(ALS) / alternative splicing / ALS
Research Abstract

We identified that caspase-3 generates two 35-kDa and 25-kDa fragments under the apoptotic condition. However, novel caspase-3-independent isoforms of these two variants were also detected under normal conditions. The 35-kDa fragment expression led to the formation of stress granules, while aggresomes were formed in the cytoplasm of cells expressing 35-kDa fragment in the presence of proteasome inhibitors.

Report

(3 results)
  • 2010 Annual Research Report   Final Research Report ( PDF )
  • 2009 Annual Research Report
  • Research Products

    (21 results)

All 2011 2010 2009 Other

All Journal Article (8 results) (of which Peer Reviewed: 4 results) Presentation (12 results) Remarks (1 results)

  • [Journal Article] マイクロRNA-206によるALSモデルマウスの疾患進行抑制と神経筋シナプス再生の促進2011

    • Author(s)
      西本祥仁
    • Journal Title

      MEDICAL BRIEFS IN Brain & Nerve 18(2)

      Pages: 8-9

    • Related Report
      2010 Final Research Report
  • [Journal Article] マイクロRNA-206によるALSモデルマウスの疾患進行抑制と神経筋シナプス再生の促進2011

    • Author(s)
      西本祥仁
    • Journal Title

      MEDICAL BRIEFS IN Brain & Nerve

      Volume: (In press)

    • Related Report
      2010 Annual Research Report
  • [Journal Article] TDP-43タンパク新規アイソフォームの同定と35-kDa封入体特性の検討2010

    • Author(s)
      西本祥仁,伊東大介,鈴木則宏
    • Journal Title

      最新医学 65(7)

      Pages: 1579-1587

    • Related Report
      2010 Final Research Report
  • [Journal Article] Novel etiological and therapeutic strategies for neurodiseases : RNA editing enzyme abnormality in sporadic amyotrophic lateral sclerosis.2010

    • Author(s)
      Hideyama T, Yamashita T, Nishimoto Y, Suzuki T, Kwak S.
    • Journal Title

      Journal of pharmacological sciences 113(1)

      Pages: 9-13

    • NAID

      10027744823

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43.2010

    • Author(s)
      Nishimoto Y, Ito D, Yagi T, Nihei Y, Tsunoda Y, Suzuki N.
    • Journal Title

      Journal of Biological Chemistry 285(1)

      Pages: 608-619

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] TDP-43タンパク新規アイソフォームの同定と35-kDa封入体特性の検討2010

    • Author(s)
      西本祥仁, 伊東大介, 鈴木則宏
    • Journal Title

      最新医学

      Volume: 65(7) Pages: 1579-1587

    • Related Report
      2010 Annual Research Report
  • [Journal Article] Novel etiological and therapeutic strategies for neurodiseases : RNA editing enzyme abnormality in sporadic amyotrophic lateral sclerosis.2010

    • Author(s)
      Hideyama T, Yamashita T, Nishimoto Y, Suzuki T, Kwak S
    • Journal Title

      Journal of pharmacological sciences

      Volume: 113(1) Pages: 9-13

    • NAID

      10027744823

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Characterization of alternative isoforms and inclusionbody of the TAR DNA-binding protein-43.2010

    • Author(s)
      Nishimoto Y, Ito D, Yagi T, Nihei Y, Tsunoda Y.Suzuki N.
    • Journal Title

      Journal of Biological Chemistry 285(1)

      Pages: 608-619

    • Related Report
      2009 Annual Research Report
    • Peer Reviewed
  • [Presentation] TDP-43蛋白の新規アイソフォームの同定とStress granuleの形成2010

    • Author(s)
      西本祥仁, 伊東大介, 鈴木則宏
    • Organizer
      第51回日本神経学会総会
    • Place of Presentation
      東京国際フォーラム
    • Year and Date
      2010-05-21
    • Related Report
      2010 Final Research Report
  • [Presentation] TDP-43蛋白の新規アイソフォームの同定とStress granuleの形成2010

    • Author(s)
      西本祥仁, 伊東大介, 鈴木則宏
    • Organizer
      第51回 日本神経学会総会
    • Place of Presentation
      東京国際フォーラム
    • Year and Date
      2010-05-21
    • Related Report
      2010 Annual Research Report
  • [Presentation] 演題「TDP-43蛋白C末断片の産生機序解明とその生化学的特性の解析」口演発表2010

    • Author(s)
      西本祥仁、伊東大介、鈴木則宏
    • Organizer
      こころの健康科学「筋萎縮性側索硬化症・認知症を伴う筋萎縮性側索硬化症・ユビキチン陽性封入体を伴う前頭側頭葉変性症脳・脊髄資源の構築」班会議
    • Place of Presentation
      東京都健康長寿医療センター高齢者ブレインバンク
    • Year and Date
      2010-01-30
    • Related Report
      2010 Final Research Report
  • [Presentation] TDP-43蛋白C末断片の産生機序解明とその生化学的特性の解析2010

    • Author(s)
      西本祥仁、伊東大介、鈴木則宏
    • Organizer
      こころの健康科学「筋萎縮性側索硬化症・認知症を伴う筋萎縮性側索硬化症・ユビキチン陽性封入体を伴う前頭側頭葉変性症 脳・脊髄資源の構築」班会議
    • Place of Presentation
      東京都健康長寿医療センター高齢者ブレインバンク
    • Year and Date
      2010-01-30
    • Related Report
      2009 Annual Research Report
  • [Presentation] 演題「Investigation into novel functions of the 35-kDa short form of TDP-432010

    • Author(s)
      西本祥仁, 伊東大介, 鈴木則宏
    • Organizer
      ポスター発表,第1回新潟大学脳研究所共同研究拠点国際シンポジウムBRI International Symposium 2010 Current Understandings and Future Directionsfor ALS
    • Place of Presentation
      新潟大学医学部統合脳機能研究センター
    • Related Report
      2010 Final Research Report
  • [Presentation] Investigation into novel functions of the 35-kDa short form of TDP-43.2010

    • Author(s)
      Yoshinori Nishimoto, Daisuke Ito, Norihiro Suzuki
    • Organizer
      BRI International Symposium 2010 Current Understandings and Future Directions for ALS
    • Place of Presentation
      新潟大学医学部 統合脳機能研究センター
    • Related Report
      2010 Annual Research Report
  • [Presentation] 「Characterization of alternative isoforms and inclusion bodies of the TDP-43」口演発表2009

    • Author(s)
      西本祥仁, 伊東大介, 八木拓也, 二瓶義廣, 角田佳子, 鈴木則宏
    • Organizer
      第10回慶應大学医科歯科大学神経内科合同カンファレンス
    • Place of Presentation
      ホテルメトロポリタンエドモント(飯田橋)
    • Year and Date
      2009-11-26
    • Related Report
      2010 Final Research Report
  • [Presentation] Characterization of alternative isoforms and inclusion bodies of the TDP-43.2009

    • Author(s)
      西本祥仁、伊東大介、八木拓也、二瓶義廣、角田佳子、鈴木則宏
    • Organizer
      第10回慶應大学医科歯科大学神経内科合同カンファルンス
    • Place of Presentation
      ホテルメトロポリタンエドモント(飯田橋)
    • Year and Date
      2009-11-26
    • Related Report
      2009 Annual Research Report
  • [Presentation] Characterization of proteolytic cleavage and inclusions of the TDP-43 protein.2009

    • Author(s)
      Yoshinori Nishimoto, Daisuke Ito, Takuya Yagi, Yoshihiro Nihei, Yoshiko Tsunoda, Norihiro Suzuki
    • Organizer
      Poster presentation, Society for Neuroscience 2009 (McCormick Place)(Abstr Soc Neurosci 2009 ; 145 : 10)
    • Place of Presentation
      Chicago, USA
    • Year and Date
      2009-10-18
    • Related Report
      2010 Final Research Report
  • [Presentation] Characterization of proteolytic cleavage and inclusions of the TDP-43 protein.2009

    • Author(s)
      Yoshinori Nishimoto, Daisuke Ito, Takuya Yagi, Yoshihiro Nihei, Yoshiko Tsunoda, Norihiro Suzuki
    • Organizer
      Society for Neuroscience 2009
    • Place of Presentation
      McCormick Place, Chicago, USA
    • Year and Date
      2009-10-18
    • Related Report
      2009 Annual Research Report
  • [Presentation] 「Characterization of alternative isoforms and inclusion bodies of the TDP-43」口演発表2009

    • Author(s)
      西本祥仁, 伊東大介, 八木拓也, 二瓶義廣, 角田佳子, 鈴木則宏
    • Organizer
      J-CAN (Japanese Consortium of Age-related Neurodegenerative disorders)
    • Place of Presentation
      東京ステーションコンファレンス(サピアタワー)
    • Year and Date
      2009-09-19
    • Related Report
      2010 Final Research Report
  • [Presentation] Characterization of alternative isoforms and inclusion bodies of the TDP-43.2009

    • Author(s)
      西本祥仁、伊東大介、八木拓也、二瓶義廣、角田佳子、鈴木則宏
    • Organizer
      J-CAN(Japanese Consortium of Age-related Neurodegenerative disorders) 2009
    • Place of Presentation
      東京ステーションコンファレンス(サピアタワー6階)
    • Year and Date
      2009-09-19
    • Related Report
      2009 Annual Research Report
  • [Remarks] ホームページ等 なし

    • Related Report
      2010 Final Research Report

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Published: 2009-04-01   Modified: 2016-04-21  

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