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Pathologcial analysis for mouse model showing dystonia

Research Project

Project/Area Number 24700351
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Nerve anatomy/Neuropathology
Research InstitutionNiigata University

Principal Investigator

HORIE Masao  新潟大学, 医歯学系, 講師 (70322716)

Project Period (FY) 2012-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥4,420,000 (Direct Cost: ¥3,400,000、Indirect Cost: ¥1,020,000)
Fiscal Year 2014: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2013: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2012: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Keywordsジストニア / ジストニン / マウス / 中枢神経系 / 末梢神経系 / モデルマウス / lacZ
Outline of Final Research Achievements

Dystonia is a disorder characterized by involuntary muscle contractions that cause slow repetitive movements, abnormal postures, or both. We had generated a novel Dystonin gene trap (DstGt) mice, in which actin-binding domain-containing isoforms are disrupted. Homozygous DstGt mice showed typical progressive neurological symptoms: severe motor disorders in their limbs and twisted postures. Electromyogram showed abnormal co-contractions of agonist and antagonist muscle in DstGt homozygotes. In histological analyses, abnormal neurofilament accumulation was observed in both peripheral and central nervous system. In order to know the abnormal neural regions possibly affecting the motor disorder in these mice, we mapped the distribution of abnormal neurofilament protein and found that it mainly concentrated in the motor-related neurons, suggesting that they are involved in the abnormal motor phenotype in DstGt.

Report

(5 results)
  • 2015 Annual Research Report   Final Research Report ( PDF )
  • 2014 Research-status Report
  • 2013 Research-status Report
  • 2012 Research-status Report
  • Research Products

    (10 results)

All 2016 2015 2014 2013

All Journal Article (1 results) (of which Peer Reviewed: 1 results) Presentation (9 results)

  • [Journal Article] Disruption of actin-binding domain-containing Dystonin protein causes dystonia musculorum in mice.2014

    • Author(s)
      Horie, M., Watanabe, K., Bepari, A. K., Nashimoto, J., Araki, K., Sano, H., Chiken, S., Nambu, A., Ono, K., Ikenaka, K., Kakita, A., Yamamura, K. and Takebayashi, H.
    • Journal Title

      Eur. J Neurosci.

      Volume: 40 Issue: 10 Pages: 3458-3471

    • DOI

      10.1111/ejn.12711

    • Related Report
      2014 Research-status Report
    • Peer Reviewed
  • [Presentation] Analyses of mouse model showing dystonia with sensory neurodegeneration2016

    • Author(s)
      堀江正男、竹林浩秀
    • Organizer
      第121回日本解剖学会総会全国学術集会
    • Place of Presentation
      ビッグパレットふくしま(福島県郡山市)
    • Year and Date
      2016-03-27
    • Related Report
      2015 Annual Research Report
  • [Presentation] Examination for possible involvement of the vestibular and reticular nuclei in the emergence for dystonia-like movements in Dystonin-deficient mice2015

    • Author(s)
      堀江正男、佐野裕美、知見聡美、小林憲太、小野勝彦、南部篤、竹林浩秀
    • Organizer
      第38回日本神経科学大会
    • Place of Presentation
      神戸国際会議場・神戸国際展示場(兵庫県神戸市)
    • Year and Date
      2015-07-21
    • Related Report
      2015 Annual Research Report
  • [Presentation] Histological analysis of the brain in Dystonin-deficient mice2015

    • Author(s)
      Masao Horie, Hiromi Sano, Satomi Chiken, Kenta Kobayashi, Katsuhiko Ono, Atsushi Nambu, Hirohide Takebayashi
    • Organizer
      第120回日本解剖学会
    • Place of Presentation
      神戸国際会議場・展示場
    • Year and Date
      2015-03-21 – 2015-03-23
    • Related Report
      2014 Research-status Report
  • [Presentation] Dystonin 変異ジストニアモデルマウスにおける ニューロン・グリアの表現型解析2015

    • Author(s)
      堀江正男
    • Organizer
      第20回グリアクラブ
    • Place of Presentation
      KKRはこだて
    • Year and Date
      2015-01-26 – 2015-01-28
    • Related Report
      2014 Research-status Report
  • [Presentation] Examination for possible involvement of the vestibular and reticular nuclei in the emergence for dystonia-like movements in Dystonin-deficient mice2014

    • Author(s)
      Masao Horie, Hiromi Sano, Satomi Chiken, Kenta Kobayashi, Katsuhiko Ono, Atsushi Nambu, Hirohide Takebayashi
    • Organizer
      第37回日本神経科学大会
    • Place of Presentation
      パシフィコ横浜
    • Year and Date
      2014-09-11 – 2014-09-13
    • Related Report
      2014 Research-status Report
  • [Presentation] Histological analysis of the brain in Dystonin-deficient mice2014

    • Author(s)
      堀江正男, 渡辺啓介, 佐野裕美, 梨本 淳一郎, 知見聡美, 南部篤, 小野勝彦, 柿田明美, 竹林浩秀
    • Organizer
      第119回日本解剖学会
    • Place of Presentation
      自治医科大学キャンパス、地域医療研修センターおよび教育・研究棟
    • Related Report
      2013 Research-status Report
  • [Presentation] Essential role of actin binding domain-containing Dystonin in the pathogenesis of dystonia musculorum2013

    • Author(s)
      堀江正男, 渡辺啓介, ベパリ オシム, 梨本 淳一郎, 荒木喜美, 小野勝彦, 池中一裕, 柿田明美, 山村研一,竹林浩秀
    • Organizer
      第37回峠の会
    • Place of Presentation
      福島県耶麻郡猪苗代町中の沢温泉 リゾートインぼなり
    • Related Report
      2013 Research-status Report
  • [Presentation] Essential role of actin binding domain-containing Dystonin in the pathogenesis of dystonia musculorum2013

    • Author(s)
      堀江正男, 渡辺啓介, ベパリ オシム, 竹林浩秀
    • Organizer
      第23回国際形態科学学会
    • Place of Presentation
      新潟市 朱鷺メッセ 新潟コンベンションセンター
    • Related Report
      2013 Research-status Report
  • [Presentation] Dystonin遺伝子改変ジストニアモデルマウスにおける原因遺伝子発現領域の解析2013

    • Author(s)
      堀江正男、渡辺啓介、Asim K Bepari、荒木善美、小野勝彦、池中一裕、竹林浩秀
    • Organizer
      第118回日本解剖学会総会・全国学術集会
    • Place of Presentation
      サンポートホール高松・かがわ国際会議場
    • Related Report
      2012 Research-status Report

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Published: 2013-05-31   Modified: 2019-07-29  

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