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The study on the role of SIP1, the causative gene for Mowat-Wilson syndrome in human, in structural and functional development of brain using mouse system

Research Project

Project/Area Number 23591525
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Pediatrics
Research InstitutionInstitute for Developmental Research, Aichi Human Service Center

Principal Investigator

HIGASHI Yujiro  愛知県心身障害者コロニー発達障害研究所, 周生期学部, 部長 (30181069)

Co-Investigator(Kenkyū-buntansha) NISHIZAKI Yuriko  愛知県心身障害者コロニー発達障害研究所, 周生期学部, リサーチレジデント (90378901)
Project Period (FY) 2011-04-28 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2012: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2011: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
KeywordsSIP1 / zeb2 / zfhx1b / モワットウィルソン症候群 / ノックアウトマウス / ZFHX1転写因子ファミリー / 脳の構造と機能 / 脳 / 精神遅滞 / 転写因子 / 遺伝・先天異常学 / モワット-ウィルソン症候群 / SIP1ノックアウトマウス / SIP1ヘテロ欠損マウス
Outline of Final Research Achievements

1) We made the SIP1-EGFP knock-in reporter mouse and analyzed the SIP1-EGFP expression pattern in detail during pre- and post-natal brain development. 2) To make the model mouse for Mowat-Wilson syndrome, we used the conditional knockout of the SIP1 gene in male germ cells to escape the difficulty in maintaining the heterozygous SIP1 knockout mice and to mimic the de novo mutation in human. We then analyzed this model mouse if they showed the relevant phenotype in relation to the symptoms in Mowat-Wilson syndrome. 3) By crossing the various Cre mouse line with SIP1 flox mouse, the SIP1 gene was deleted in the specific regions or cells in mouse brain, and we analyzed the resulting phenotype from the morphological and functional viewpoints.

Report

(5 results)
  • 2014 Annual Research Report   Final Research Report ( PDF )
  • 2013 Research-status Report
  • 2012 Research-status Report
  • 2011 Research-status Report
  • Research Products

    (6 results)

All 2014 2012 Other

All Journal Article (3 results) (of which Peer Reviewed: 3 results) Presentation (3 results)

  • [Journal Article] SIP1 expression patterns in brain investigated by generating a SIP1-EGFP reporter knock-in mouse.2014

    • Author(s)
      Nishizaki, Y., Takagi, T., Matsui, F. and Higashi, Y.
    • Journal Title

      Genesis

      Volume: 52 Issue: 1 Pages: 56-67

    • DOI

      10.1002/dvg.22726

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Dlx1&2-dependent expression of Zfhx1b (Sip1, Zeb2) regulates the fate switch between cortical and striatal interneurons.2012

    • Author(s)
      McKinsey GL, Lindtner S, Trzcinski B, Visel A, Pennacchio LA, Huylebroeck D, Higashi Y, Rubenstein JL.
    • Journal Title

      Neuron

      Volume: 77 Pages: 83-98

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Dual-Mode Modulation of Smad Signaling by Smad-Interacting Protein Sip1 Is Required for Myelination in the Central Nervous System2012

    • Author(s)
      Weng, Q. 他 15名
    • Journal Title

      Neuron

      Volume: 73 Pages: 713-728

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Presentation] de novo型Sip1ヘテロ変異マウスを用いたMowat-Wilson syndrome のモデル動物としての有用性の検討2012

    • Author(s)
      高木豪、東雄二郎
    • Organizer
      第35回 日本分子生物学会
    • Place of Presentation
      福岡国際会議場
    • Related Report
      2012 Research-status Report
  • [Presentation] モデルマウスを用いたモワット・ウィルソン症候群の病態形成の解析

    • Author(s)
      高木豪、西崎有利子、松井ふみ子、東雄二郎
    • Organizer
      第36回日本分子生物学会
    • Place of Presentation
      神戸国際会議場(神戸)
    • Related Report
      2013 Research-status Report
  • [Presentation] レポーターノックインマウスを用いたモワット-ウィルソン症候群の原因遺伝子SIP1の発現解析

    • Author(s)
      西崎有利子、高木豪、松井ふみ子、東雄二郎
    • Organizer
      第36回日本分子生物学会
    • Place of Presentation
      神戸国際会議場(神戸)
    • Related Report
      2013 Research-status Report

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

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