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analysis of the pathomechanism of amyotrophic lateral sclerosis-focusing on Cajal bodies

Research Project

Project/Area Number 22790814
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeSingle-year Grants
Research Field Neurology
Research InstitutionNiigata University

Principal Investigator

YOKOSEKI Akio  新潟大学, 医歯学系, 特任准教授 (90515719)

Project Period (FY) 2010 – 2011
Project Status Completed (Fiscal Year 2011)
Budget Amount *help
¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2011: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2010: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
KeywordsALS / TDP-43 / Cajal小体 / coilin / Coilin
Research Abstract

TAR DNA binding protein of 43 kDa(TDP-43) is a major protein of ubiquitinated cytoplasmic inclusions in glia and neurons in amyotrophic lateral sclerosis(ALS). TDP-43 has been reported to associate with nuclear bodies : Cajal bodies, GEM bodies, PML, and SC35. Therefore, we focused on Cajal bodies. The mean number of Cajal bodies in sporadic ALS cases was statistically decreased than that in controls. Cajal bodies play an important function as maturation of small nuclear and nucleolar ribonucleoproteins. Taken together, neuronal death may be caused by dysregulation of transcription in ALS motor neurons.

Report

(3 results)
  • 2011 Annual Research Report   Final Research Report ( PDF )
  • 2010 Annual Research Report
  • Research Products

    (12 results)

All 2011 2010 Other

All Journal Article (5 results) (of which Peer Reviewed: 4 results) Presentation (2 results) Book (2 results) Remarks (3 results)

  • [Journal Article] Onodera O, Genotype-phenotype correlations in early onset ataxia with ocular motor apraxia and hypoalbuminemia2011

    • Author(s)
      Yokoseki A, Ishihara T, Koyama A, Shiga A, Yamada M, Suzuki C, Sekijima Y, Maruta K, Tsuchiya M, Date H, Sato T, Tada M, Ikeuchi T, Tsuji S, Nishizawa M
    • Journal Title

      Brain

      Volume: 134 Pages: 1387-1399

    • Related Report
      2011 Final Research Report
    • Peer Reviewed
  • [Journal Article] Cerebral small-vessel disease protein HTRA1 controls the amount of TGF-beta1 via clevage of proTGF-beta12011

    • Author(s)
      Shiga A, Nozaki H, Yokoseki A, Nihonmatsu M, Kawata H, Kato T, Koyama A, Arima K, Ikeda M, Katada S, Toyoshima Y, Takahashi H, Tanaka A, Nakano I, Ikeuchi T, Nishizawa M, Onodera O
    • Journal Title

      Hum Mol Genet

      Volume: 20 Pages: 1800-1810

    • Related Report
      2011 Final Research Report
    • Peer Reviewed
  • [Journal Article] Genotype-phenotype correlations in early onset ataxia with ocular motorapraxia and hypoalbuminemia2011

    • Author(s)
      Yokoseki A, Ishihara T, Koyama A, et al
    • Journal Title

      Brain

      Volume: 134 Issue: 5 Pages: 1387-1399

    • DOI

      10.1093/brain/awr069

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Cerebral small-vessel disease protein HTRA1 controls the amount of TGF-β1 via cleavage of proTGF-β1.2011

    • Author(s)
      Shiga A
    • Journal Title

      Hum Mol Genet.

      Volume: 20 Issue: 9 Pages: 1800-1810

    • DOI

      10.1093/hmg/ddr063

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] 【神経変性疾患におけるTDP-43】家族性筋萎縮性側索硬化症とTDP-432010

    • Author(s)
      石原智彦, 横関明男, 西澤正豊, 高橋均, 小野寺理
    • Journal Title

      最新医学

      Volume: 65 Pages: 1648-1653

    • Related Report
      2011 Final Research Report
  • [Presentation] TDP-43関連ALSの脊髄前角細胞におけるCajal小体の減少,神経変性疾患に関する調査研究班2011

    • Author(s)
      横関明男,譚春鳳,志賀篤,石原智彦,有泉優子,佐藤達哉,高橋均,西澤正豊,小野寺理
    • Organizer
      平成23年度班会議
    • Place of Presentation
      東京都
    • Year and Date
      2011-12-17
    • Related Report
      2011 Final Research Report
  • [Presentation] TDP-43関連ALSの脊髄前角細胞におけるCajal小体の減少2011

    • Author(s)
      横関明男, 譚春鳳, 志賀篤, 石原智彦, 有泉優子, 佐藤達哉, 高橋均, 西澤正豊, 小野寺理
    • Organizer
      神経変性疾患に関する調査研究班平成23年度班会議
    • Place of Presentation
      都市センターホテル(東京都)
    • Year and Date
      2011-12-17
    • Related Report
      2011 Annual Research Report
  • [Book] 高次脳機能障害Q & A基礎編「TDP-43という言葉をよく聞きますが,これは何のことなのでしょうか?」2011

    • Author(s)
      横関明男,石原智彦,西澤正豊,小野寺理
    • Publisher
      新興医学出版社
    • Related Report
      2011 Final Research Report
  • [Book] TDP-43という言葉をよく聞きますが,これは何のことなのでしょうか?(高次脳機能障害Q&A基礎編)2011

    • Author(s)
      横関明男, 石原智彦, 西澤正豊, 小野寺理
    • Publisher
      新興医学出版社
    • Related Report
      2011 Annual Research Report
  • [Remarks]

    • URL

      http://www.bri.niigata-u.ac.jp/~neuroweb/laboratory/research_basic_002.html

    • Related Report
      2011 Final Research Report
  • [Remarks]

    • URL

      http://www.bri.niigata-u.ac.jp/~neuroweb/laboratory/research_basic_002.html

    • Related Report
      2011 Annual Research Report
  • [Remarks]

    • URL

      http://www.bri.niigata-u.ac.jp/~neuroweb/laboratory/research_basic_002.html

    • Related Report
      2010 Annual Research Report

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Published: 2010-08-23   Modified: 2016-04-21  

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