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Intracellular Reaction of Skeletal Muscle in Myasthenia Gravis

Research Project

Project/Area Number 25461273
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Neurology
Research InstitutionKanazawa University

Principal Investigator

Iwasa Kazuo  金沢大学, 医学系, 准教授 (10345613)

Co-Investigator(Renkei-kenkyūsha) YOSHIKAWA Hiroaki  金沢大学, 保健管理センター, 教授 (10272981)
Research Collaborator FURUKAWA Yutaka  
MOTOZAKI Yuko  
Project Period (FY) 2013-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2015: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2014: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Fiscal Year 2013: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Keywords重症筋無力症 / 抗アセチルコリン受容体抗体 / 骨格筋 / 神経筋接合部 / 小胞体ストレス応答 / カベオリン3 / 骨格筋再生機能 / 骨格筋免疫応答 / カベオリン3蛋白 / 骨格筋再生機構 / 骨格筋修復再生機能 / 骨格筋内オルガネラ / ミトコンドリア / 補体
Outline of Final Research Achievements

In myasthenia gravis (MG), intracellular reaction of muscle was important for pathology and medication. We evaluated GRP78 (ER stress protein) upregulation in muscle with MG. ER stress in muscle is important for repair after injury. Upregulation of GRP78 in MG muscle plays an important role in muscle repair and improvement. An increase of GRP78 also plays immunoregulatory roles in MG, such as serving as a defense mechanism against immunological attack and modulating the immune system itself. Some MG muscles showed patchy/scarce caveolin-3 distribution. Partial loss of caveolin-3 expression was relatively common in MG. Caveolin-3 levels were higher in MG vs. non-myogenic patient. Caveolin-3 upregulation may be required for restoring MG muscle.
In summary, MG might have damage not only in the neuromuscular junction, but also in muscle intracellular areas. Our findings may help to understand the muscle response in MG. However, more studies are needed for elucidating the mechanisms.

Report

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

    (8 results)

All 2016 2015 2014

All Journal Article (1 results) (of which Peer Reviewed: 1 results) Presentation (7 results) (of which Int'l Joint Research: 1 results)

  • [Journal Article] Increased skeletal muscle expression of the endoplasmic reticulum chaperone GRP78 in patients with myasthenia gravis.2014

    • Author(s)
      Iwasa K, Nambu Y, Motozaki Y, Furukawa Y, Yoshikawa H, Yamada M.
    • Journal Title

      J Neuroimmunol.

      Volume: 273 Issue: 1-2 Pages: 72-76

    • DOI

      10.1016/j.jneuroim.2014.05.006

    • Related Report
      2014 Research-status Report
    • Peer Reviewed
  • [Presentation] The partial loss and irregular overexpression of caveolin-3 in muscle cell membrane is detected in myasthenia gravis patients2016

    • Author(s)
      Kazuo Iwasaa, Yutaka Furukawa, Hiroaki Yoshikawa, Masahito Yamada
    • Organizer
      Myology 2016 - 5th International Congress of Myology
    • Place of Presentation
      Lyon Convention Centre, Lyon, Flance
    • Year and Date
      2016-03-14
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Presentation] 重症筋無力症における骨格筋融合シグナル蛋白mRNAの発現亢進について2015

    • Author(s)
      岩佐和夫、吉川弘明、古川裕、山田正仁
    • Organizer
      第27回日本神経免疫学会
    • Place of Presentation
      長良川国際会議、岐阜
    • Year and Date
      2015-09-15
    • Related Report
      2015 Annual Research Report
  • [Presentation] 重症筋無力症骨格筋におけるcaveolin-3の発現について2015

    • Author(s)
      岩佐和夫、吉川弘明、古川裕、山田正仁
    • Organizer
      第56回日本神経学会学術大会
    • Place of Presentation
      朱鷺メッセ( 新潟コンベンションセンター)、新潟
    • Year and Date
      2015-05-20 – 2015-05-23
    • Related Report
      2014 Research-status Report
  • [Presentation] 重症筋無力症骨格筋におけるcaveolin-3の発現について2015

    • Author(s)
      岩佐和夫、吉川弘明、古川裕、山田正仁:
    • Organizer
      第56回日本神経学会学術大会
    • Place of Presentation
      朱鷺メッセ、新潟
    • Year and Date
      2015-05-20
    • Related Report
      2015 Annual Research Report
  • [Presentation] 重症筋無力症骨格筋におけるcaveolin-3の発現について2014

    • Author(s)
      岩佐和夫、吉川弘明、古川裕、山田正仁
    • Organizer
      第26回神経免疫学会
    • Place of Presentation
      金沢歌劇座、金沢
    • Year and Date
      2014-09-04 – 2014-09-06
    • Related Report
      2014 Research-status Report
  • [Presentation] Increased skeletal muscle expression of the endoplasmic reticulum chaperone GRP78 in patients with myasthenia gravis2014

    • Author(s)
      Iwasa K, Nambu Y, Motozaki Y, Furukawa Y, Yoshikawa H, Yamada M
    • Organizer
      13th International Congress on Neuromuscular Disease
    • Place of Presentation
      ACROPOLIS CONVENTION CENTRE, Nice, France
    • Year and Date
      2014-07-05 – 2014-07-10
    • Related Report
      2014 Research-status Report
  • [Presentation] Increased skeletal muscle expression of the endoplasmic reticulum chaperone GRP78 in patients with myasthenia gravis2014

    • Author(s)
      岩佐 和夫
    • Organizer
      13th International Congress on Neuromuscular Disease
    • Place of Presentation
      Nice, France
    • Related Report
      2013 Research-status Report

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Published: 2014-07-25   Modified: 2019-07-29  

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