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Role of fukutin-dependent glycosylation of dystroglycan on skeletal muscle development, maintenance, and muscular dystrophy pathogenesis

Research Project

Project/Area Number 21790318
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeSingle-year Grants
Research Field Pathological medical chemistry
Research InstitutionKobe University

Principal Investigator

KANAGAWA Motoi  Kobe University, 大学院・医学研究科, 助教 (00448044)

Project Period (FY) 2008 – 2010
Project Status Completed (Fiscal Year 2010)
Budget Amount *help
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2010: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2009: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
Keywordsフクチン / ジストログリカン / 福山型筋ジストロフィー / 糖鎖異常 / 筋形成維持 / 筋形成・維持
Research Abstract

Fukuyama-type muscular dystrophy, which is caused by mutations in the fukutin gene, is a severe congenital form of muscular dystrophy. Pathogenesis of this disorder is poorly understood and currently no effective treatment is available. In this study, we generated conditional knock-out mice that lack fukutin selectively in the skeletal muscle. These mice showed pathology similar to Fukuyama-type muscular dystrophy, and thus would be a useful model for understanding molecular pathogenesis and establishing therapeutic strategy such as gene therapy.

Report

(3 results)
  • 2010 Annual Research Report   Final Research Report ( PDF )
  • 2009 Annual Research Report
  • Research Products

    (33 results)

All 2010 2009 2008 Other

All Journal Article (8 results) (of which Peer Reviewed: 8 results) Presentation (22 results) Remarks (3 results)

  • [Journal Article] Post-translational maturation of dystroglycan is necessary for pikachurin binding and ribbon synaptic localization.2010

    • Author(s)
      Kanagawa M, Omori Y, Sato S, Kobayashi K, Miyagoe-Suzuki Y, Takeda S, Endo T, Furukawa T, Toda T.
    • Journal Title

      J.Biol.Chem. 285

      Pages: 31208-31216

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Residual glycosylation of dystroglycan in model mice to Fukuyama-type congenital muscular dystrophy is sufficient to maintain voluntary exercise activity and skeletal muscle regeneration capability.2010

    • Author(s)
      Lu Z, Kanagawa M, Toda T.
    • Journal Title

      Medical Journal Osaka University 53

      Pages: 51-61

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Post-translational maturation of dystroglycan is necessary for pikachurin binding and ribbon synaptic localization.2010

    • Author(s)
      Motoi Kanagawa
    • Journal Title

      J Biol Chem

      Volume: 285 Pages: 31208-31216

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Residual glycosylation of dystroglycan in model mice to Fukuyama-type congenital muscular dystrophy is sufficient to maintain voluntary exercise activity and skeletal muscle regeneration capability2010

    • Author(s)
      Zhongpeng Lu
    • Journal Title

      Medical Journal Osaka University

    • Related Report
      2009 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy.2009

    • Author(s)
      Kanagawa M, Nishimoto A, Chiyonobu T, Takeda S, Miyagoe-SuzukiY, Wang F, Fujikake N, Taniguchi M, Lu Z, Tachikawa M, Nagai Y, Tashiro F, Miyazaki J, Tajima Y, Takeda S, Endo T, Kobayashi K, Campbell KP, Toda T.
    • Journal Title

      Hum.Mol.Genet. 18

      Pages: 621-631

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan.2009

    • Author(s)
      Han R, Kanagawa M, Yoshida-Moriguchi T, Rader EP, Ng RA, Michele DE, Muirhead DE, Kunz S, Moore SA, Iannaccone ST, Miyake K, McNeil PL, Mayer U, Oldstone MB, Faulkner JA, Campbell KP.
    • Journal Title

      Proc.Natl.Acad.Sci.USA. 106

      Pages: 12573-12579

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Pikachurin, a dystroglycan ligand, is essential for photoreceptor ribbon synapse formation.2008

    • Author(s)
      Sato S, Omori Y, Katoh K, Kondo M, Kanagawa M, Miyata K, Funabiki K, Koyasu T, Kajimura N, Miyoshi T, Sawai H, Kobayashi K, Tani A, Toda T, Usukura J, Tano Y, Fujikado T, Furukawa T.
    • Journal Title

      Nat.Neurosci. 11

      Pages: 923-931

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Reduced expression of sarcospan in muscles of Fukuyama congenital muscular dystrophy.2008

    • Author(s)
      Wakayama Y, Inoue M, Kojima H, Yamashita S, Shibuya S, Jimi T, Hara H, Matsuzaki Y, Oniki H, Kanagawa M, Kobayashi K, Toda T.
    • Journal Title

      Histol.Histopathol. 23

      Pages: 1425-1438

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Presentation] Molecular basis and physiological roles of dystroglycan glycosylation and its relevance to diseases2010

    • Author(s)
      Kanagawa M
    • Organizer
      第33回日本分子生物学会年会 第83回日本生化学会大会 合同大会
    • Place of Presentation
      神戸
    • Year and Date
      2010-12-10
    • Related Report
      2010 Final Research Report
  • [Presentation] Molecular basis and physiological roles of dystroglycan glycosylation and its relevance to diseases2010

    • Author(s)
      Motoi Kanagawa
    • Organizer
      第33回日本分子生物学会年会・第83回日本生化学会大会合同大会
    • Place of Presentation
      神戸国際展示場(兵庫県)(招待講演)
    • Year and Date
      2010-12-10
    • Related Report
      2010 Annual Research Report
  • [Presentation] レトロトランスポゾン挿入変異をもつ福山型先天性筋ジストロフィーモデルマウスの創出と糖鎖遺伝子治療2010

    • Author(s)
      金川基
    • Organizer
      日本人類遺伝学会第55回大会
    • Place of Presentation
      さいたま.
    • Year and Date
      2010-10-29
    • Related Report
      2010 Final Research Report
  • [Presentation] レトロトランスポゾン挿入変異をもつ福山型先天性筋ジストロフィーモデルマウスの創出と糖鎖遺伝子治療2010

    • Author(s)
      金川基
    • Organizer
      日本人類遺伝学会第55回大会
    • Place of Presentation
      大宮ソニックシティ(埼玉県)
    • Year and Date
      2010-10-29
    • Related Report
      2010 Annual Research Report
  • [Presentation] Disruption of dystroglycan-pikachurin interaction underlies the molecular pathogenesis of eye abnormalities in dystroglycanopathy.2010

    • Author(s)
      Kanagawa M
    • Organizer
      15th International Congress of the World Muscle Society.
    • Place of Presentation
      Kumamoto, Japan.
    • Year and Date
      2010-10-13
    • Related Report
      2010 Final Research Report
  • [Presentation] Disruption of dystroglycan-pikachurin interaction underlies the molecular pathogenesis of eye abnormalities in dystroglycanopathy2010

    • Author(s)
      Motoi Kanagawa
    • Organizer
      The 15th International Congress of World Muscle Society
    • Place of Presentation
      崇城大学市民ホール(熊本県)
    • Year and Date
      2010-10-13
    • Related Report
      2010 Annual Research Report
  • [Presentation] Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy.2010

    • Author(s)
      Kanagawa M
    • Organizer
      25th International Carbohydrate Symposium ICS2010.
    • Place of Presentation
      Makuhari, Japan.
    • Year and Date
      2010-08-03
    • Related Report
      2010 Final Research Report
  • [Presentation] Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy2010

    • Author(s)
      Motoi Kanagawa
    • Organizer
      The 25th International Carbohydrate Symposium
    • Place of Presentation
      幕張メッセ(千葉県)
    • Year and Date
      2010-08-03
    • Related Report
      2010 Annual Research Report
  • [Presentation] Disruption of dystroglycan-pikachurin interaction underlies the molecular pathogenesis of eye abnormalities in dystroglycanopathy.2010

    • Author(s)
      Kanagawa M
    • Organizer
      第28回内藤コンファレンス Glycan Expression and Regulation [I] : Functions and Disease mechanisms(糖鎖の発現と制御[I]-機能から病態まで-)
    • Place of Presentation
      葉山
    • Year and Date
      2010-07-28
    • Related Report
      2010 Final Research Report
  • [Presentation] Post-translational maturation of dystroglycan is necessary for pikachurin binding and ribbon synaptic localization2010

    • Author(s)
      Motoi Kanagawa
    • Organizer
      第28回内藤コンファレンス
    • Place of Presentation
      湘南国際村センター(神奈川県)
    • Year and Date
      2010-07-28
    • Related Report
      2010 Annual Research Report
  • [Presentation] Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy.2010

    • Author(s)
      Kanagawa M
    • Organizer
      New Directions in Biology and Disease of Skeletal Muscle.
    • Place of Presentation
      Ottawa, Canada
    • Year and Date
      2010-05-06
    • Related Report
      2010 Final Research Report
  • [Presentation] Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy2010

    • Author(s)
      Motoi Kanagawa
    • Organizer
      The Ottawa Conference on New Directions in Biology and Disease of Skeletal Muscle
    • Place of Presentation
      オタワ(カナダ)
    • Year and Date
      2010-05-06
    • Related Report
      2010 Annual Research Report
  • [Presentation] Generation of a model mouse for Fukuyama-type Congenital Muscular Dystrophy.2009

    • Author(s)
      Sato K
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      横浜
    • Year and Date
      2009-12-10
    • Related Report
      2010 Final Research Report
  • [Presentation] 骨格筋特異的fukutin KOマウスの表現型解析~福山型先天性筋ジストロフィーの病態解明にむけて~2009

    • Author(s)
      佐藤佳乃子
    • Organizer
      第32回 日本分子生物学会大会
    • Place of Presentation
      パシフィコ横浜(神奈川県)
    • Year and Date
      2009-12-10
    • Related Report
      2009 Annual Research Report
  • [Presentation] Essential role of dystroglycan in the maintenance of cell membrane.2009

    • Author(s)
      Kanagawa M
    • Organizer
      第82回日本生化学会大会
    • Place of Presentation
      神戸
    • Year and Date
      2009-10-24
    • Related Report
      2010 Final Research Report
  • [Presentation] Involvement of dysferlin-mediated membrane repair system in progression of glycosylation-defect muscular dystrophy.2009

    • Author(s)
      Lu Z
    • Organizer
      第82回日本生化学会大会
    • Place of Presentation
      神戸
    • Year and Date
      2009-10-24
    • Related Report
      2010 Final Research Report
  • [Presentation] Subcellular localization and POMGnT1-binding of fukutin missense mutants which are involved in the onset of FCMD.2009

    • Author(s)
      Kanagawa M
    • Organizer
      第82回日本生化学会大会
    • Place of Presentation
      神戸
    • Year and Date
      2009-10-24
    • Related Report
      2010 Final Research Report
  • [Presentation] Involvement of dysferlin-mediated membrane repair system in progression of glycosylation-defect muscular dystrophy2009

    • Author(s)
      Zhongpeng Lu
    • Organizer
      第82回 日本生化学会大会
    • Place of Presentation
      神戸国際展示場(兵庫県)
    • Year and Date
      2009-10-24
    • Related Report
      2009 Annual Research Report
  • [Presentation] FCMD発症にかかわるフクチンミスセンス変異体の細胞内局在とPOMGnT1結合の観察2009

    • Author(s)
      立川雅司
    • Organizer
      第82回 日本生化学会大会
    • Place of Presentation
      神戸国際展示場(兵庫県)
    • Year and Date
      2009-10-24
    • Related Report
      2009 Annual Research Report
  • [Presentation] ジストログリカンの糖鎖異常を示すモデルマウスを用いた先天性筋ジストロフィーの病態解析と治療戦略の構築2008

    • Author(s)
      金川基
    • Organizer
      第31回日本分子生物学会年会 第81回日本生化学会大会 合同大会
    • Place of Presentation
      神戸
    • Year and Date
      2008-12-09
    • Related Report
      2010 Final Research Report
  • [Presentation] ジストログリカンの糖鎖異常を伴う先天性筋ジストロフィーの病態と治療戦略2008

    • Author(s)
      金川基
    • Organizer
      第28回日本糖質科学会
    • Place of Presentation
      つくば
    • Year and Date
      2008-08-18
    • Related Report
      2010 Final Research Report
  • [Presentation] Generation of a model mouse for Fukuyama congenital muscular dystrophy carrying a retrotransposal insertion in the 3' UTR in the fukutin gene.2008

    • Author(s)
      Kanagawa M
    • Organizer
      The Third New Directions in Skeletal Muscle Biology Meeting.
    • Place of Presentation
      New Orleans, U.S.A.
    • Year and Date
      2008-04-29
    • Related Report
      2010 Final Research Report
  • [Remarks] ホームページ等

    • URL

      http://www.med.kobe-u.ac.jp/clgene/

    • Related Report
      2010 Final Research Report
  • [Remarks]

    • URL

      http://www.med.kobe-u.ac.jp/clgene/

    • Related Report
      2010 Annual Research Report
  • [Remarks]

    • URL

      http://www.med.kobe-u.ac.jp/clgene/

    • Related Report
      2009 Annual Research Report

URL: 

Published: 2009-04-01   Modified: 2016-04-21  

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