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RNA metabolism and RNA-binding proteins in polyglutamine diseases

Research Project

Project/Area Number 23791007
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Neurology
Research InstitutionThe Institute of Physical and Chemical Research

Principal Investigator

KINO Yoshihiro  独立行政法人理化学研究所, 脳科学総合研究センター, 客員研究員 (80415140)

Project Period (FY) 2011 – 2012
Project Status Completed (Fiscal Year 2013)
Budget Amount *help
¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000)
Fiscal Year 2012: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2011: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Keywords神経変性疾患 / ポリグルタミン / RNA結合タンパク質 / 凝集体 / リピート伸長疾患
Research Abstract

Polyglutamine diseases, including Huntington's disease, are genetic disorders caused by a repeat expansion of CAG repeat tracts in protein-coding regions. This study revealed aberrant RNA processing in polyglutamine diseases using cultured cells and disease model mice, suggesting the involvement of RNA-binding proteins in the disease pathogenesis. In addition, RNA-binding proteins have a potential regulatory role in the expression of the gene products of a polyglutamine disease.

Report

(4 results)
  • 2013 Annual Research Report   Final Research Report ( PDF )
  • 2012 Research-status Report
  • 2011 Research-status Report
  • Research Products

    (21 results)

All 2014 2013 2012 2011 Other

All Journal Article (9 results) (of which Peer Reviewed: 3 results) Presentation (10 results) (of which Invited: 1 results) Book (2 results)

  • [Journal Article] Dexamethasone induces heat shock response and slows down disease progression in mouse and fly models of Huntington's disease2014

    • Author(s)
      Maheshwari M, Bhutani S, Das A, Mukherjee R, Sharma A, Kino Y, Nukina N, Jana NR
    • Journal Title

      Human Molecular Genetics

      Volume: 23(10) Pages: 2737-2751

    • Related Report
      2013 Final Research Report
  • [Journal Article] Cyclin-dependent kinase 5 phosphorylates and induces the degradation of ataxin-22014

    • Author(s)
      Asada, A., Yamazaki, R., Kino, Y., Saito, T., Kimura, T., Miyake, M., Hasegawa, M., Nukina, N. & Hisanaga, S.
    • Journal Title

      Neurosci. Lett.

      Volume: 563 Pages: 112-117

    • DOI

      10.1016/j.neulet.2014.01.046

    • Related Report
      2013 Annual Research Report 2013 Final Research Report
    • Peer Reviewed
  • [Journal Article] FUS/TLS医学のあゆみ医学2014

    • Author(s)
      紀嘉浩、貫名信行
    • Journal Title

      医療のいまがわかるキーワード2014

      Volume: 249(5) Pages: 438-438

    • Related Report
      2013 Final Research Report
  • [Journal Article] Dexamethasone induces heat shock response and slows down disease progression in mouse and fly models of Huntington's disease2014

    • Author(s)
      Maheshwari, M., Bhutani, S., Das, A., Mukherjee, R., Sharma, A., Kino, Y., Nukina, N. & Jana, N.R.
    • Journal Title

      Hum. Mol. Genet.

      Volume: 23 Issue: 10 Pages: 2737-2751

    • DOI

      10.1093/hmg/ddt667

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed
  • [Journal Article] FUS/TLS2014

    • Author(s)
      紀 嘉浩、貫名 信行
    • Journal Title

      医学のあゆみ 医学・医療のいまがわかるキーワード 2014

      Volume: 249 Pages: 438-438

    • Related Report
      2013 Annual Research Report
  • [Journal Article] ハンチントン病の治療法開発とモデルマウスを用いた評価実験医学 in vivo2012

    • Author(s)
      紀嘉浩、貫名信行
    • Journal Title

      実験医学によるサイエンスと疾患解明

      Volume: Vol. 30 No. 2 (増刊) Pages: 203-210

    • Related Report
      2013 Final Research Report
  • [Journal Article] ハンチントン病の治療法開発とモデルマウスを用いた評価2012

    • Author(s)
      紀 嘉浩、貫名 信行
    • Journal Title

      実験医学増刊号「in vivo実験医学によるヒト疾患解明の最前線」

      Volume: 30 Pages: 203-210

    • Related Report
      2011 Research-status Report
  • [Journal Article] Alternative splicing of myomesin 1 gene is aberrantly regulated in myotonic dystrophy type 12011

    • Author(s)
      Koebis M, Ohsawa N, Kino Y, Sasagawa N, Nishino I, Ishiura S
    • Journal Title

      Genes to Cells

      Volume: 16(9) Pages: 961-972

    • Related Report
      2013 Final Research Report
  • [Journal Article] Alternative splicing of myomesin 1 gene is aberrantly regulated in myotonic dystrophy type 1.2011

    • Author(s)
      Koebis M, Ohsawa N, Kino Y, Sasagawa N, Nishino I, Ishiura S.
    • Journal Title

      Genes to Cells

      Volume: 16(9) Issue: 9 Pages: 961-972

    • DOI

      10.1111/j.1365-2443.2011.01542.x

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Presentation] Protein/RNA aggregation in neurological disorders2013

    • Author(s)
      Kino Y, Nukina N
    • Organizer
      Neuro2013
    • Place of Presentation
      京都
    • Year and Date
      2013-06-21
    • Related Report
      2013 Final Research Report
  • [Presentation] Nuclear localization of MBNL1 : Implications for splicing-mediated autoregulation and expanded repeat-related protein expression2011

    • Author(s)
      Kino Y, Washizu C, Kurosawa M, Oma Y, Ishiura S, Nukina N
    • Organizer
      第34回日本分子生物学会年会
    • Place of Presentation
      横浜
    • Year and Date
      2011-12-13
    • Related Report
      2013 Final Research Report
  • [Presentation] Muscleblind proteins repress aberrant protein expression derived from expanded repeats2011

    • Author(s)
      Kino Y, Washizu C, Kurosawa M, Oma Y, Ishiura S, Nukina N
    • Organizer
      8th International Myotonic Dystrophy Consortium Meeting
    • Place of Presentation
      タンパ(米国)
    • Year and Date
      2011-12-01
    • Related Report
      2013 Final Research Report
  • [Presentation] RNA結合タンパク質MBNL1によるリピート伸長疾患RNAの制御2011

    • Author(s)
      紀嘉浩、鷲頭知花、黒澤大、貫名信行
    • Organizer
      包括型脳科学推進支援ネットワーク夏のワークショップ
    • Place of Presentation
      神戸
    • Year and Date
      2011-08-23
    • Related Report
      2013 Final Research Report
  • [Presentation] 筋萎縮性側索硬化症に関連する変異はFUS/TLSの細胞内局在およびスプライシング制御能に影響する2011

    • Author(s)
      紀嘉浩、鷲頭知花、奥野弥佐子、黒澤大、山田みず樹、土井宏、貫名信行
    • Organizer
      第52回日本神経学会学術大会
    • Place of Presentation
      名古屋
    • Year and Date
      2011-05-18
    • Related Report
      2013 Final Research Report
  • [Presentation] 筋萎縮性側索硬化症に関連する変異はFUS/TLSの細胞内局在およびスプライシング制御に影響する2011

    • Author(s)
      紀嘉浩、鷲頭知花、奥野弥佐子、黒沢大、山田みず樹、土井宏、貫名信行
    • Organizer
      第52回日本神経学会学術大会
    • Place of Presentation
      名古屋
    • Related Report
      2011 Research-status Report
  • [Presentation] RNA結合タンパク質MBNL1によるリピート伸長疾患RNAの制御2011

    • Author(s)
      紀 嘉浩、鷲頭 知花、黒澤 大、貫名 信行
    • Organizer
      包括型脳科学研究推進支援ネットワーク 夏のワークショップ
    • Place of Presentation
      神戸
    • Related Report
      2011 Research-status Report
  • [Presentation] Muscleblind proteins repress aberrant protein expression derived from expanded repeats2011

    • Author(s)
      Yoshihiro Kino, Chika Washizu, Masaru Kurowasa, Yoko Oma, Shoichi Ishiura, Nobuyuki Nukina
    • Organizer
      8th International Myotonic Dystrophy Consortium Meeting
    • Place of Presentation
      タンパ
    • Related Report
      2011 Research-status Report
  • [Presentation] Nuclear Localization of MBNL1: Implications for splicing-mediated autoregulation and expanded repeat-related protein expression2011

    • Author(s)
      Yoshihiro Kino, Chika Washizu, Masaru Kurosawa, Yoko Oma, Shoichi Ishiura, Nobuyuki Nukina
    • Organizer
      第34回日本分子生物学会年会
    • Place of Presentation
      横浜
    • Related Report
      2011 Research-status Report
  • [Presentation] Protein/RNA aggregation in neurological disorders

    • Author(s)
      Yoshihiro Kino, Nobuyuki Nukina
    • Organizer
      Neuro2013
    • Place of Presentation
      京都
    • Related Report
      2012 Research-status Report
    • Invited
  • [Book] モデル動物実験マニュアル疾患モデルの作成と利用脳・神経疾患第2節ハンチントン病モデルマウス2011

    • Author(s)
      紀嘉浩、黒澤大、貫名信行(分担執筆:三品昌美編)
    • Publisher
      株式会社エル・アイ・シー
    • Related Report
      2013 Final Research Report
  • [Book] モデル動物実験マニュアル 疾患モデルの作成と利用 脳・神経疾患 (第2節 ハンチントン病モデルマウス)2011

    • Author(s)
      紀 嘉浩、黒澤 大、貫名 信行 (分担執筆・三品昌美編)
    • Publisher
      株式会社 エル・アイ・シー
    • Related Report
      2011 Research-status Report

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Published: 2011-08-05   Modified: 2019-07-29  

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