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characterization of the laminin-dystroglycan interaction in neuropathy and its therapeutic application

Research Project

Project/Area Number 23591256
Research Category

Grant-in-Aid for Scientific Research (C)

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

Principal Investigator

SAITO Fumiaki  帝京大学, 医学部, 准教授 (40286993)

Co-Investigator(Kenkyū-buntansha) MASAKI Toshihiro  帝京科学大学, 医療科学部, 教授 (00585028)
HAGIWARA Hiroki  帝京科学大学, 医療科学部, 教授 (80276732)
Co-Investigator(Renkei-kenkyūsha) MATSUMURA Kiichiro  帝京大学, 医学部, 教授 (50260922)
Project Period (FY) 2011 – 2013
Project Status Completed (Fiscal Year 2013)
Budget Amount *help
¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
Fiscal Year 2013: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2012: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2011: ¥2,340,000 (Direct Cost: ¥1,800,000、Indirect Cost: ¥540,000)
Keywords神経分子病態学 / ニューロパチー / ジストログリカン / 髄鞘形成 / 骨格筋再生 / Large / 筋再生 / LARGE
Research Abstract

The interaction between dystroglycan in Schwann cell and laminin in the basement membrane is crucial for the myelination of peripheral nerve. We generated LARGE transgenic mice in which this interaction is greatly enhanced. In the peripheral nerves of these mice, the expression of some genes related to myelination was altered. We crossed LARGE transgenic mice with dy2J mice, which exhibit dysmyelination of the peripheral nerve, however the defects in myelination were not ameliorated. Since the peripheral nerve affects the regeneration of the skeletal muscle via neuromuscular junction, we examined the regeneration of dy2J mice skeletal muscles. We demonstrated that the muscle regeneration was suppressed by the overexpression of LARGE in these mice.

Report

(4 results)
  • 2013 Annual Research Report   Final Research Report ( PDF )
  • 2012 Research-status Report
  • 2011 Research-status Report
  • Research Products

    (28 results)

All 2014 2013 2012 2011 Other

All Journal Article (9 results) (of which Peer Reviewed: 7 results,  Open Access: 1 results) Presentation (17 results) Book (2 results)

  • [Journal Article] Overexpression of LARGE suppresses muscle regeneration via down-regulation of insulin-like growth factor 1 and aggravates muscular dystrophy in mice2014

    • Author(s)
      Saito F, Kanagawa M, Ikeda M, Hagiwara H, Masaki T, Ohkuma H, Katanosaka Y, Shimizu T, Sonoo M, Toda T, Matsumura K.
    • Journal Title

      Hum Mol Genet

      Volume: 23 Issue: 17 Pages: 4543

    • DOI

      10.1093/hmg/ddu168

    • Related Report
      2013 Final Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Overexpression of LARGE suppresses muscle regeneration via down-regulation of insulin-like growth factor 1 and aggravates muscular dystrophy in mice2014

    • Author(s)
      Saito F, Kanagawa M, Ikeda M, Hagiwara H, Masaki T, Ohkuma H, Shimizu T, Sonoo M, Toda T, Matsumura K.
    • Journal Title

      Hum Mol Genet

      Volume: in press

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed
  • [Journal Article] LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy2013

    • Author(s)
      Goddeeris MM, Wu B, Venzke D, Yoshida-Moriguchi T, Saito F, Matsumura K, Moore SA, Campbell KP
    • Journal Title

      Nature

      Volume: 503 Issue: 7474 Pages: 136

    • DOI

      10.1038/nature12605

    • URL

      https://pure.teikyo.jp/en/publications/293f859d-c603-49a0-aa0f-5aeb574731fc

    • Related Report
      2013 Annual Research Report 2013 Final Research Report
    • Peer Reviewed
  • [Journal Article] LARGEによるα-ジストログリカノパチーに対する治療戦略2012

    • Author(s)
      斉藤史明
    • Journal Title

      神経内科

      Volume: 76 Pages: 367-371

    • Related Report
      2013 Final Research Report
    • Peer Reviewed
  • [Journal Article] Histone deacetylase inhibitor trichostatin A enhances myogenesis by coordinating muscle regulatory factors and myogenic repressors2011

    • Author(s)
      Hagiwara H, Saito F, Masaki T, Ikeda M, Nakamura-Ohkuma A, Shimizu T, Matsumura K
    • Journal Title

      Biochem Biophys Res Commun

      Volume: 414 Issue: 4 Pages: 826

    • DOI

      10.1016/j.bbrc.2011.10.036

    • Related Report
      2013 Final Research Report 2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Secretion of N-terminal domain of α-dystroglycan in cerebrospinal fluid2011

    • Author(s)
      Saito F, Saito-Arai Y, Nakamura-Okuma A, Ikeda M, Hagiwara H, Masaki T, Shimizu T, Matsumura K
    • Journal Title

      Biochem Biophys Res Commun

      Volume: 411 Issue: 2 Pages: 365

    • DOI

      10.1016/j.bbrc.2011.06.150

    • Related Report
      2013 Final Research Report 2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Fukuyama-type congenital muscular dystrophy and defective glycosylation of α-dystroglycan2011

    • Author(s)
      Saito F, Matsumura K
    • Journal Title

      Skeleta Muscle

      Volume: 1 Issue: 1

    • DOI

      10.1186/2044-5040-1-22

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Largeによるα-ジストログリカノパチーに対する治療法の開発2011

    • Author(s)
      斉藤史明
    • Journal Title

      臨床神経学

      Volume: 51 Pages: 918-921

    • Related Report
      2011 Research-status Report
  • [Journal Article] α-ジストログリカンの機能異常と筋ジストロフィー2011

    • Author(s)
      斉藤史明,松村喜一郎
    • Journal Title

      生体の科学

      Volume: 62 Pages: 88-90

    • Related Report
      2011 Research-status Report
  • [Presentation] 骨格筋特異的Fukutin欠損マウスに対するLARGEの過剰発現の影響2013

    • Author(s)
      斉藤史明,金川基,萩原宏毅,真先敏弘,池田美樹,清水輝夫,園生雅弘,戸田達史,松村喜一郎
    • Organizer
      第54回日本神経学会学術大会
    • Place of Presentation
      東京
    • Year and Date
      2013-05-31
    • Related Report
      2013 Final Research Report
  • [Presentation] レスベラトロールの先天性筋ジストロフィーモデルマウス対する効果の検討2013

    • Author(s)
      萩原宏毅,斉藤史明,真先敏弘,清水輝夫,松村喜一郎,園生雅弘
    • Organizer
      第54回日本神経学会学術大会
    • Place of Presentation
      東京
    • Year and Date
      2013-05-31
    • Related Report
      2013 Final Research Report
  • [Presentation] Effects of overexpression of LARGE on a mouse model of congenital muscular dystrophy2012

    • Author(s)
      Saito F, Hagiwara H, Ikeda M, Masaki T, Shimizu T, Matsumura K
    • Organizer
      17th International congress of the World Muscle Society
    • Place of Presentation
      Perth, Australia
    • Year and Date
      2012-10-09
    • Related Report
      2013 Final Research Report
  • [Presentation] LARGEによるα-dystroglycanの機能亢進を用いた先天性筋シストロフィー治療の試み2012

    • Author(s)
      斉藤史明,萩原宏毅,真先敏弘,池田美樹,園生雅弘,松村喜一郎
    • Organizer
      第53回日本神経学会学術大会
    • Place of Presentation
      東京
    • Year and Date
      2012-05-24
    • Related Report
      2013 Final Research Report
  • [Presentation] 転写因子Pax3はMyoDと細胞周期の制御因子に作用して筋分化を抑制する2012

    • Author(s)
      萩原宏毅,斉藤史明,真先敏弘,清水輝夫,松村喜一郎,園生雅弘
    • Organizer
      第53回日本神経学会学術大会
    • Place of Presentation
      東京
    • Year and Date
      2012-05-24
    • Related Report
      2013 Final Research Report
  • [Presentation] 細胞外H1蛋白は末梢神経・胸腺における新しいラミニン結合蛋白てある2012

    • Author(s)
      真先敏弘,斉藤史明,萩原宏毅,松村喜一郎,園生雅弘
    • Organizer
      第53回日本神経学会学術大会
    • Place of Presentation
      東京
    • Year and Date
      2012-05-24
    • Related Report
      2013 Final Research Report
  • [Presentation] Dystroglycanの機能亢進を用いた癌細胞抑制に関する検討2011

    • Author(s)
      斉藤史明,萩原宏毅,池田美樹,黄山,真先敏弘,松村喜一郎
    • Organizer
      第84回日本生化学会大会
    • Place of Presentation
      京都
    • Year and Date
      2011-09-22
    • Related Report
      2013 Final Research Report
  • [Presentation] シンポジウム筋ジストロフィー新規治療法開発の最前線.Largeによるα-ジストログリカノパチーに対する治療法の開発2011

    • Author(s)
      斉藤史明
    • Organizer
      第52回日本神経学会学術大会
    • Place of Presentation
      名古屋
    • Year and Date
      2011-05-19
    • Related Report
      2013 Final Research Report
  • [Presentation] Largeによるα-dystroglycanの機能修復と疾患治療への応用2011

    • Author(s)
      斉藤史明,萩原宏毅,大熊文美,池田美樹,真先敏弘,清水輝夫,松村喜一郎
    • Organizer
      第52回日本神経学会学術大会
    • Place of Presentation
      名古屋
    • Year and Date
      2011-05-19
    • Related Report
      2013 Final Research Report
  • [Presentation] ヒストン脱アセチル化酵素阻害剤は筋原性制御因子に作用して骨格筋分化を促進する2011

    • Author(s)
      萩原宏毅,斉藤史明,真先敏弘,大熊文美,松村喜一郎,清水輝夫
    • Organizer
      第52回日本神経学会学術大会
    • Place of Presentation
      名古屋
    • Year and Date
      2011-05-18
    • Related Report
      2013 Final Research Report
  • [Presentation] シンポジウム 筋ジストロフィー新規治療法開発の最前線.Largeによるα-ジストログリカノパチーに対する治療法の開発2011

    • Author(s)
      斉藤史明
    • Organizer
      第52回日本神経学会学術大会(招待講演)
    • Place of Presentation
      名古屋
    • Related Report
      2011 Research-status Report
  • [Presentation] Largeによるα-dystroglycanの機能修復と疾患治療への応用2011

    • Author(s)
      斉藤史明、萩原宏毅、大熊文美、池田美樹、真先敏弘、清水輝夫、松村喜一郎
    • Organizer
      第52回日本神経学会学術大会
    • Place of Presentation
      名古屋
    • Related Report
      2011 Research-status Report
  • [Presentation] 骨格筋特異的Fukutin欠損マウスに対するLARGEの過剰発現の影響

    • Author(s)
      斉藤史明,金川基,萩原宏毅,真先敏弘,池田美樹,清水輝夫,園生雅弘,戸田達史,松村喜一郎
    • Organizer
      第54回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Annual Research Report
  • [Presentation] レスベラトロールの先天性筋ジストロフィーモデルマウス対する効果の検討

    • Author(s)
      萩原宏毅,斉藤史明,真先敏弘,清水輝夫,松村喜一郎,園生雅弘
    • Organizer
      第54回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2013 Annual Research Report
  • [Presentation] Effects of overexpression of LARGE on a mouse model of congenital muscular dystrophy

    • Author(s)
      Saito F, Hagiwara H, Ikeda M, Masaki T, Shimizu T, Matsumura K.
    • Organizer
      17th International congress of the World Muscle Society
    • Place of Presentation
      Perth, Australia
    • Related Report
      2012 Research-status Report
  • [Presentation] The role of the transcriptional factor Pax3 on myogenesis and the effect on the expression of myogenic regulatory factor

    • Author(s)
      Hagiwara H, Shan H, Masaki T, Ikeda M, Shimizu T, Matsumura K, Saito F.
    • Organizer
      17th International congress of the World Muscle Society
    • Place of Presentation
      Perth, Australia
    • Related Report
      2012 Research-status Report
  • [Presentation] LARGE による α!dystroglycan の機能亢進を用いた先天性筋シストロフィー治療の試み

    • Author(s)
      斉藤史明,萩原宏毅,真先敏弘,池田美樹,園生雅弘,松村喜一郎
    • Organizer
      第53回日本神経学会学術大会
    • Place of Presentation
      東京
    • Related Report
      2012 Research-status Report
  • [Book] 筋強直性ジストロフィー.In :神経疾患最新の治療2012-20142012

    • Author(s)
      斉藤史明,清水輝夫
    • Publisher
      南江堂
    • Related Report
      2013 Final Research Report
  • [Book] 筋強直性ジストロフィー In: 神経疾患最新の治療 2012-20142012

    • Author(s)
      斉藤史明,清水輝夫
    • Publisher
      南江堂
    • Related Report
      2011 Research-status Report

URL: 

Published: 2011-08-05   Modified: 2019-07-29  

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