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Structure of nuclear envelope and Nuclear calcium transient of Emery Dreifuss muscular dystrophy

Research Project

Project/Area Number 17K16244
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Pediatrics
Research InstitutionKanazawa University

Principal Investigator

Shimojima Masaya  金沢大学, 附属病院, 特任助教 (00613754)

Research Collaborator Yamagishi Masakazu  
Hayashi Kenshi  
Yoshida Shohei  
Okada Hirofumi  
Project Period (FY) 2017-04-01 – 2019-03-31
Project Status Completed (Fiscal Year 2018)
Budget Amount *help
¥2,730,000 (Direct Cost: ¥2,100,000、Indirect Cost: ¥630,000)
Fiscal Year 2018: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2017: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Keywordsエメリードレフュス型筋ジストロフィー / ラミン / エメリン / 心筋症 / 核膜陥入層 / 核内カルシウム / ネスプリン / 核内カルシウム動態 / エメリーフドレフュス症候群 / 脳・神経 / 神経筋疾患 / 循環器 / 核膜陥入 / 小児科
Outline of Final Research Achievements

Emery-Dreifuss muscular dystrophy (EDMD) is caused by nuclear envelope protein such as emerin, lamin and nesprin. To examine the role of lamin, emerin and nesprin in cardiomyocyte, we produced EDMD-iPS derived cardiomyocyte, rat neonate cardiomyocyte during siRNA-mediated lamin, emerin and nesprin.
The incidence of cardiomyocyte with nuclear invagination was significantly decreased and the nuclear size was significantly increased. These data suggest that lamin has a pivotal function in the maintenance of nuclear invagination and nuclear size. To clarify mechanism of EDMD, the expression of pERK and HDAC3 was assesed. Although the expression of pERK was not changed significantly, the distribution of HDAC3 in nuclear was decreased under horse serum condition.

Academic Significance and Societal Importance of the Research Achievements

エメリードレフュス型筋ジストロフィー(EDMD)のメカニズムについては、様々な研究報告がなされているが、エメリンやラミンといった核蛋白の異常が原因であることを除いてあまり解明されていない。今回、エメリンが欠損したときと同様にラミンノックダウン時にも核の構造変化をきたすこと、また、HDAC3のようなヒストン脱アセチル酵素の分布の変化もきたしていることを示唆されるような結果が得られたことより、HDAC3によって制御されるシグナルの更なる解析をすすめることによって、EDMDの病態解明及び創薬につながっていく可能性があると考えられる。

Report

(3 results)
  • 2018 Annual Research Report   Final Research Report ( PDF )
  • 2017 Research-status Report
  • Research Products

    (2 results)

All 2018 2017

All Journal Article (1 results) Presentation (1 results) (of which Int'l Joint Research: 1 results)

  • [Journal Article] エメリンが核膜陥入層及び,核内カルシウム動態に重要な役割を果たす2018

    • Author(s)
      下島正也
    • Journal Title

      金沢大学十全医学雑誌

      Volume: 127 Pages: 49-50

    • NAID

      120006534710

    • Related Report
      2018 Annual Research Report
  • [Presentation] Emery Dreifuss Muscular Dystrophy is caused by change of nuclear invagination and calcium transient.2017

    • Author(s)
      Masaya Shimojima
    • Organizer
      Amrican Heart Association 2017
    • Related Report
      2017 Research-status Report
    • Int'l Joint Research

URL: 

Published: 2017-04-28   Modified: 2020-03-30  

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