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2011 Fiscal Year Final Research Report

Elucidation of pathogenic mechanisms of myasthenia gravis caused by MuSK antibodies using a new synchronized experimental animal model

Research Project

  • PDF
Project/Area Number 21591102
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionTokyo Metropolitan Geriatric Hospital and Institute of Gerontology

Principal Investigator

SHIGEMOTO Kazuhiro  地方独立行政法人東京都健康長寿医療センター(東京都健康長寿医療センター研究所), 東京都健康長寿医療センター研究所, 研究部長 (40284400)

Co-Investigator(Renkei-kenkyūsha) KONISHI Tetsuro  独立行政法人国立病院機構宇多野病院, 神経内科, 院長 (50426508)
Project Period (FY) 2009 – 2011
Keywords重症筋無力症 / 神経筋接合部 / MuSK / 動物疾患モデル / 治療薬
Research Abstract

We generated a new animal model in which 100% of mice synchronously develop experimental animal model of myasthenia gravis after immunization with MuSK protein. Our model provides a valuable platform from which to evaluate the role of MuSK signaling in NMJ maintenance and the immune mechanisms and pathophysiology of MuSK-MG, and we propose that 3, 4-DAP may be effective as a symptomatic therapy.

  • Research Products

    (28 results)

All 2012 2011 2010 2009 Other

All Journal Article (11 results) (of which Peer Reviewed: 4 results) Presentation (13 results) Book (1 results) Remarks (3 results)

  • [Journal Article] 3, 4-Diaminopyridine improves neuromuscular transmission in a MuSK antibody-induced mouse model of myasthenia gravis2012

    • Author(s)
      Mori, S., Kishi, M., Kubo, S., Akiyoshi, T., Yamada, S., Miyazaki, T., Konishi, T., Maruyama, N., and Shigemoto, K
    • Journal Title

      Journal of neuroimmunology

      Volume: 245 Pages: 75-78

    • DOI

      DOI:10.1016/j.jneuroim.2012.02.010

    • Peer Reviewed
  • [Journal Article] Antibodies against muscle-specific kinase impair both presynaptic and postsynaptic functions in a murine model of myasthenia gravis2012

    • Author(s)
      Mori, S., Kubo, S., Akiyoshi, T., Yamada, S., Miyazaki, T., Hotta, H., Desaki, J., Kishi, M., Konishi, T., Nishino, Y., Miyazawa, A., Maruyama, N., and Shigemoto, K
    • Journal Title

      The American journal of pathology

      Volume: 180 Pages: 798-810

    • DOI

      DOI:10.1016/j.ajpath.2011.10.031

    • Peer Reviewed
  • [Journal Article] Divalent and monovalent autoantibodies cause dysfunction of MuSK by distinct mechanisms in a rabbit model of myasthenia gravis2012

    • Author(s)
      Mori, S., Yamada, S., Kubo, S., Chen, J., Matsuda, S., Shudou, M., Maruyama, N., and Shigemoto, K
    • Journal Title

      Journal of neuroimmunology

      Volume: 244 Pages: 1-7

    • DOI

      doi:10.1016/j.jneuroim.2011.12.005

    • Peer Reviewed
  • [Journal Article] サルコペニアの発症機構2011

    • Author(s)
      重本和宏,森秀一,宮崎剛,久保幸穂
    • Journal Title

      神経内科

      Volume: 75(6) Pages: 584-588

  • [Journal Article] シナプスを介したサルコペニアの発症メカニズムとバイオマーカー2011

    • Author(s)
      重本和宏,森秀一、宮崎剛、久保幸穂
    • Journal Title

      日本老年医学会雑誌

      Volume: 48(1) Pages: 42-43

  • [Journal Article] 運動神経細胞とサルコペニア2011

    • Author(s)
      重本和宏、森秀一、宮崎剛、久保幸穂
    • Journal Title

      Modern Physician

      Volume: 131 Pages: 1308-11

  • [Journal Article] Muscle weakness and neuromuscular junctions in aging and disease2010

    • Author(s)
      Shigemoto, K., Kubo, S., Mori, S., Yamada, S., Akiyoshi, T., and Miyazaki, T
    • Journal Title

      Geriatr Gerontol Int

      Volume: 10 Pages: 137-147

    • DOI

      DOI:10.1111/j.1447-0594.2010.00608.x

    • Peer Reviewed
  • [Journal Article] 抗MuSK抗体2010

    • Author(s)
      大田潔江、大田光煕,重本和宏
    • Journal Title

      日本臨床

      Volume: 68 Pages: 633-6

  • [Journal Article] 神経筋接合部の異常と筋萎縮2010

    • Author(s)
      重本和宏,森秀一,久保幸穂,山田茂,秋好沢諭,宮崎剛
    • Journal Title

      メジカルビュー社.老年医学

      Pages: 135-144

  • [Journal Article] 神経筋接合部位の異常と筋力低下2009

    • Author(s)
      重本和宏、久保幸穂、丸山直記
    • Journal Title

      日本老年医学会雑誌

      Volume: 46(2) Pages: 106-113

  • [Journal Article] サルコペニア(加齢性筋肉減少症)とそのバイオマーカー2009

    • Author(s)
      重本和宏、丸山直記
    • Journal Title

      老年医学

      Volume: 47(6) Pages: 733-735

  • [Presentation] A new synchronized model of myasthenia gravis caused by anti-MuSK antibodies2012

    • Author(s)
      Shigemoto, K
    • Organizer
      International Conference on Nervous System Autoimmunity
    • Place of Presentation
      Bangalore, India
    • Year and Date
      20120127-30
  • [Presentation] 抗MuSK抗体陽性重症筋無力症の発症メカニズムに基づく治療薬の検討2012

    • Author(s)
      森秀一,久保幸穂,岸雅彦,小西哲郎,重本和宏
    • Organizer
      第53回日本神経学会学術大会
    • Place of Presentation
      東京
    • Year and Date
      2012-05-25
  • [Presentation] Distinct mechanisms between divalent and monovalent MuSK antibodies involved with dysfunction of MuSK leading to myasthenia gravis2011

    • Author(s)
      Mori S, Kubo S, Yamada S, Miyazaki T, Shigemoto K
    • Organizer
      Society for Neuroscience
    • Place of Presentation
      Washington, D. C
    • Year and Date
      20111112-16
  • [Presentation] サルコペニアのメカニズム解明に向けて2011

    • Author(s)
      重本和宏
    • Organizer
      第33回日本基礎老化学会シンポジウム
    • Place of Presentation
      野田
    • Year and Date
      20111008-09
  • [Presentation] MuSKとDok-7の相互作用は神経筋シナプスの維持に重要である2011

    • Author(s)
      森秀一,重本和宏
    • Organizer
      第66回日本体力医学会大会
    • Place of Presentation
      下関
    • Year and Date
      2011-09-17
  • [Presentation] Distinct mechanisms between divalent and monovalent MuSK antibodies involved with dysfunction of MuSK leading to myasthenia gravis2011

    • Author(s)
      Mori S, Kubo S, Yamada S, Miyazaki T, Shigemoto K
    • Organizer
      第34回日本神経科学会大会
    • Place of Presentation
      横浜
    • Year and Date
      2011-09-15
  • [Presentation] Complement activation is dispensable for myasthenia gravis caused by MuSK antibodies2010

    • Author(s)
      Shigemoto K, Mori S, Kubo S, Akiyoshi T, Yamada S, Miyazaki T
    • Organizer
      10th International Congress of Neuroimmunology
    • Place of Presentation
      Barcelona, Spain
    • Year and Date
      20100000
  • [Presentation] Strategy to treatment of myasthenia gravis caused by MuSK antibodies using a synchronized experimental animal model2010

    • Author(s)
      Mori S, Kubo S, Akiyoshi T, Yamada S, Miyazaki T, Shigemoto K
    • Organizer
      Society for Neuroscience
    • Place of Presentation
      San Diego
    • Year and Date
      2010-11-17
  • [Presentation] New strategy to treatment of myasthenia gravis caused by MuSK antibodies using a synchronized experimental animal model2010

    • Author(s)
      Mori S, Kubo S, Akiyoshi T, Yamada S, Miyazaki T, Shigemoto K
    • Organizer
      第33回日本神経科学会大会
    • Place of Presentation
      神戸
    • Year and Date
      2010-09-03
  • [Presentation] 筋は逆行性シグナルにより筋・運動神経を維持する2010

    • Author(s)
      森秀一,久保幸穂,秋好沢諭,山田茂,宮崎剛,重本和宏
    • Organizer
      第33回日本基礎老化学会大会
    • Place of Presentation
      名古屋
    • Year and Date
      2010-06-18
  • [Presentation] Activation of complement is indispensable for myasthenia gravis caused by MuSK antibodies2009

    • Author(s)
      Mori S, Kubo S, Akiyoshi T, Yamada S, Miyazaki T, Shigemoto K
    • Organizer
      Society for Neuroscience
    • Place of Presentation
      Chicago
    • Year and Date
      2009-10-17
  • [Presentation] Activation of complement is indispensable for myasthenia gravis caused by MuSK antibodies2009

    • Author(s)
      Mori S, Kubo S, Akiyoshi T, Yamada S, Shigemoto K
    • Organizer
      第32回日本神経科学大会
    • Place of Presentation
      名古屋
    • Year and Date
      2009-09-19
  • [Presentation] MuSKは神経筋シナプスの維持に重要である.研究モデル動物の確立2009

    • Author(s)
      森秀一,久保幸穂,秋好沢諭,山田茂,宮崎剛,重本和宏
    • Organizer
      第32回日本基礎老化学会大会
    • Place of Presentation
      横浜
    • Year and Date
      2009-06-27
  • [Book] The Immunopathogenesis of Experimental Autoimmune Myasthenia Gravis Induced by Autoantibodies against Muscle-specific kinase. Book title : Myasthenia gravis Diseas Mechanisms and Immune Intervention. Editor : Premkumar Christadoss2009

    • Author(s)
      Shigemoto, K. Kubo, S. Mori. S. Yamada, S. Miyazaki, T. Akiyoshi, T and Maruyama, S
    • Total Pages
      304-323
    • Publisher
      Linus Publication, Inc.
  • [Remarks] The Jackson Laboratory, USA

    • URL

      http://jaxmice.jax.org/news/2012/Myasthenia_gravis.html

  • [Remarks] The Jackson Laboratory, USA Podcast

    • URL

      http://jaxmice.jax.org/multimedia/podcasts/20120524-Shigemoto.mp3

  • [Remarks] 東京都健康長寿医療センター研究所老年病研究チーム運動器医学

    • URL

      http://www.tmghig.jp/J_TMIG/j_research/DA33.html

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Published: 2013-07-31  

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