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Novel pathogenesis of myasthenia gravis: immune checkpoint molecules and complements regulatory molecules and their development into therapy

Research Project

Project/Area Number 21K07421
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Review Section Basic Section 52020:Neurology-related
Research InstitutionIshikawa Prefectural Nursing University

Principal Investigator

Iwasa Kazuo  石川県立看護大学, 看護学部, 教授 (10345613)

Co-Investigator(Kenkyū-buntansha) 吉川 弘明  金沢大学, 保健管理センター, 教授 (10272981)
古川 裕  金沢大学, 附属病院, 助教 (50881033)
野崎 一朗  金沢大学, 附属病院, 助教 (60736495)
Project Period (FY) 2021-04-01 – 2024-03-31
Project Status Completed (Fiscal Year 2023)
Budget Amount *help
¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000)
Fiscal Year 2023: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2022: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2021: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Keywords重症筋無力症 / 骨格筋 / 補体制御因子 / 免疫チェックポイント分子 / 新規病態 / 免疫チェックポイント因子 / 免疫調整因子
Outline of Research at the Start

重症筋無力症は、運動ニューロンと骨格筋との連絡の場(神経筋接合部)における骨格筋側の情報受容器(アセチルコリン受容体)に対し自ら抗体を作成し(自己免疫疾患)、易疲労性などの筋力低下をきたす神経難病の一つである。MGの治療法に関する研究は日進月歩でおこなわれているが、本研究では自らの身体に備わる自己免疫機能の調節機構がMGの病態に関与していることを証明し、その機構を利用した新たな治療法を開発することを目的としている。

Outline of Final Research Achievements

In this study, we demonstrated that the expression of the complement regulatory proteins in the skeletal muscle of myasthenia gravis was involved in the pathogenesis of the disease. The expression of complement regulatory proteins in skeletal muscle was found to be clustered at the neuromuscular junction. In myasthenia gravis, a negative correlation was found between the expression level of the complement regulatory proteins and the clinical symptom score. This indicates that the expression of the complement regulatory proteins at the neuromuscular junction of skeletal muscle is involved in the pathogenesis of myasthenia gravis and provides a new insight into the pathogenesis of myasthenia gravis.

Academic Significance and Societal Importance of the Research Achievements

本研究の成果は重症筋無力症の骨格筋において、病態に関わる神経筋接合部の破壊を制御する補体制御因子の発見となった。この発見は、重症筋無力症の新たな病態の理解を深めたのみでなく、新規治療法の開発につながることが期待でき、今後の治療に向けた更なる研究が望まれる。

Report

(4 results)
  • 2023 Annual Research Report   Final Research Report ( PDF )
  • 2022 Research-status Report
  • 2021 Research-status Report
  • Research Products

    (5 results)

All 2023 2022

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

  • [Journal Article] CD59 Expression in Skeletal Muscles and Its Role in Myasthenia Gravis2023

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

      Neurology Neuroimmunology and Neuroinflammation

      Volume: 10(1) Issue: 1

    • DOI

      10.1212/nxi.0000000000200057

    • Related Report
      2023 Annual Research Report 2022 Research-status Report
    • Peer Reviewed / Open Access
  • [Presentation] 重症筋無力症患者骨格筋における補体制御因子発現に関する検討2023

    • Author(s)
      岩佐和夫、古川裕、吉川弘明、山田正仁、小野賢二郎
    • Organizer
      第64回日本神経学会学術大会
    • Related Report
      2023 Annual Research Report
  • [Presentation] CD59 cluster at the neuromuscular junction in skeletal muscles and its role in Myasthenia Gravis2023

    • Author(s)
      Kazuo Iwasa,Yutaka Furukawa,Hiroaki Yoshikawa,Masahito Yamada,Kenjiro Ono
    • Organizer
      26th World Congress of Neurology
    • Related Report
      2023 Annual Research Report
    • Int'l Joint Research
  • [Presentation] 重症筋無力症患者骨格筋における補体制御因子発現2023

    • Author(s)
      岩佐和夫
    • Organizer
      第9回日本筋学会学術集会および第10回筋ジストロフィー医療研究会合同開催
    • Related Report
      2023 Annual Research Report
    • Invited
  • [Presentation] 重症筋無力症骨格筋における補体制御因子発現に関する検討2022

    • Author(s)
      岩佐和夫, 古川裕, 吉川弘明, 山田正仁, 小野賢二郎
    • Organizer
      第34回日本神経免疫学会学術集会
    • Related Report
      2022 Research-status Report

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Published: 2021-04-28   Modified: 2025-01-30  

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