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Clarify the pathogenic mechnism of microlissencephaly

Research Project

Project/Area Number 15K08283
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field General medical chemistry
Research InstitutionOsaka City University

Principal Investigator

Jin Mingyue  大阪市立大学, 大学院医学研究科, 講師 (60740404)

Co-Investigator(Renkei-kenkyūsha) HIROTSUNE Shinji  大阪市立大学, 大学院医学研究科, 教授 (80337526)
MATSUMOTO Sakiko  大阪市立大学, 大学院医学研究科, 助教 (00789654)
YAMADA Masami  福井大学, 学術研究院医学系部門, 教授 (10322851)
YASUNAGA Takuo  九州工業大学, 大学院情報工学研究院, 教授 (60251394)
MIYADA Takaki  名古屋大学, 医学系研究科, 教授 (70311751)
OIWA Kazuhiro  国立研究開発法人情報通信研究機構, 未来ICT研究所, 主管研究員 (10211096)
FURUTA Ken'ya  国立研究開発法人情報通信研究機構, 研究員 (40571831)
TOBA Shiori  弘前医療福祉大学, 短期大学部, 教授 (40419891)
Research Collaborator MIYAZAWA Atsuo  
NAGAI Kakeharu  
REVERSADE Bruno  
POMP Oz  
Project Period (FY) 2015-04-01 – 2018-03-31
Project Status Completed (Fiscal Year 2017)
Budget Amount *help
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2017: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2016: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2015: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Keywords中枢神経形成不全 / カタニンp80 / 細胞質ダイニン / NuMA / 微小管ネットワーク / α-Synuclein / microlissencephaly / 神経幹細胞の分裂 / 神経細胞の遊走 / 神経幹細胞の増殖 / 滑脳症・小脳症
Outline of Final Research Achievements

In this project, we performed functional analyses of katanin p80, a pathogenic causing gene of microlissencephaly. This congenital neurodevelopmental disorder has the clinical features of both microcephaly and lissencephaly. To date, mutations on the genes correlated with proliferation of neural progenitors cause microcephaly, and associated with the neuronal migration induces lissencephaly. This highlight critical functions for katanin p80 during neurogenesis and neuronal migration, suggesting the existence of a common pathophysiological pathway responsible for microcephaly and lissencephaly. In this study, we demonstrated that katanin p80 regulates microtubule (MT) remodeling with NuMA (Nuclear Mitotic Apparatus protein) and cytoplasmic dynein. Our findings provide valuable insights into the pathogenesis of severe microlissencephaly, in which p80 and NuMA delineate a common pathway for neurogenesis and neuronal migration via microtubule organization at the centrosome/spindle pole.

Report

(4 results)
  • 2017 Annual Research Report   Final Research Report ( PDF )
  • 2016 Research-status Report
  • 2015 Research-status Report
  • Research Products

    (8 results)

All 2017 2016 2015 Other

All Int'l Joint Research (2 results) Journal Article (2 results) (of which Int'l Joint Research: 1 results,  Peer Reviewed: 2 results,  Open Access: 2 results,  Acknowledgement Compliant: 1 results) Presentation (4 results) (of which Int'l Joint Research: 3 results,  Invited: 1 results)

  • [Int'l Joint Research] Institute of Medical Biology/Human Genetics and Embryology Laboratory(シンガポール)

    • Related Report
      2016 Research-status Report
  • [Int'l Joint Research] Institute of Medical Biology(Singapore)

    • Related Report
      2015 Research-status Report
  • [Journal Article] Alpha-synuclein facilitates to form short unconventional microtubules that have a unique function in the axonal transport2017

    • Author(s)
      Toba Shiori、Jin Mingyue、Yamada Masami、Kumamoto Kanako、Matsumoto Sakiko、Yasunaga Takuo、Fukunaga Yuko、Miyazawa Atsuo、Fujita Sakiko、Itoh Kyoko、Fushiki Shinji、Kojima Hiroaki、Wanibuchi Hideki、Arai Yoshiyuki、Nagai Takeharu、Hirotsune Shinji
    • Journal Title

      Sci Rep

      Volume: 7 Issue: 1 Pages: 16386-16386

    • DOI

      10.1038/s41598-017-15575-3

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Katanin p80, NuMA and cytoplasmic dynein cooperate to control microtubule dynamics2017

    • Author(s)
      Jin M., Pomp O., Shinoda T., Toba S., Torisawa T., Furuta K., Oiwa K., Yasunaga T., Kitagawa D., Matsumura S., Miyata T., Tan T., Reversade B. and Hirotsune S.
    • Journal Title

      Scientific Reports

      Volume: 7

    • NAID

      120005997787

    • Related Report
      2016 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research / Acknowledgement Compliant
  • [Presentation] Alpha-synuclein has a unique function in axonal transport2017

    • Author(s)
      Mingyue Jin, Shiori Toba, Sakiko Matsumoto, Shinji Hirotsune
    • Organizer
      The 12th Biennial Conference of Chinese Neuroscience Society
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Katanin p80 regulates microtubule organization with NuMA and cytoplasmic dynein during brain development2016

    • Author(s)
      Jin M., Pomp O., Toba S., Tan T., Reversade B. and Hirotsune S.
    • Organizer
      World life science conference 2016
    • Place of Presentation
      国家会議センター(北京市、中国)
    • Year and Date
      2016-11-01
    • Related Report
      2016 Research-status Report
    • Int'l Joint Research
  • [Presentation] カタニンp80による微小管ネットワーク構築の制御2016

    • Author(s)
      金 明月、Oz Pomp、篠田友康、鳥羽栞、鳥澤嵩行、古田健也、大岩和弘、安永卓生、北川大樹、宮田卓樹、Bruno Reversade、広常真治
    • Organizer
      生体運動研究合同班会議2016
    • Place of Presentation
      キャンパスプラザ京都(京都府、京都市)
    • Year and Date
      2016-01-08
    • Related Report
      2015 Research-status Report
  • [Presentation] Katanin p80 regulates microtubule organizations via cytoplasmic dynein during brain development2015

    • Author(s)
      Mingyue Jin, Oz Pomp, Bruno Reversade and Shinji Hirotsune
    • Organizer
      9th Asian biophysics Association Symposium 2015
    • Place of Presentation
      Hangzhou (China)
    • Year and Date
      2015-05-09
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research / Invited

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Published: 2015-04-16   Modified: 2019-03-29  

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