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Structural, functional, and pathological studies on a novel "post-phosphoryl sugar chain"

Research Project

Project/Area Number 24687017
Research Category

Grant-in-Aid for Young Scientists (A)

Allocation TypePartial Multi-year Fund
Research Field Functional biochemistry
Research InstitutionKobe University

Principal Investigator

KANAGAWA Motoi  神戸大学, 医学(系)研究科(研究院), 講師 (00448044)

Project Period (FY) 2012-04-01 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥27,300,000 (Direct Cost: ¥21,000,000、Indirect Cost: ¥6,300,000)
Fiscal Year 2014: ¥8,060,000 (Direct Cost: ¥6,200,000、Indirect Cost: ¥1,860,000)
Fiscal Year 2013: ¥10,270,000 (Direct Cost: ¥7,900,000、Indirect Cost: ¥2,370,000)
Fiscal Year 2012: ¥8,970,000 (Direct Cost: ¥6,900,000、Indirect Cost: ¥2,070,000)
Keywords糖鎖 / 筋ジストロフィー / ジストログリカン / 遺伝子治療 / 筋再生 / フクチン / 翻訳後修飾
Outline of Final Research Achievements

This study aimed at understanding of structure, modification mechanism, and physiological roles of novel post-translational modification termed “post-phosphoryl sugar chain”. We also examined the pathogenesis of diseases caused by defects in post-phosphoryl modification in order to develop therapeutic strategies. Structural analysis on unique recombinant glycoprotein and functional analysis of the disease gene products opened a new roadmap toward full understanding of the molecular basis of post-phosphoryl modification. Analysis on disease mouse models revealed a new pathogenesis of muscular dystrophy, which led us to propose an effective therapeutic strategy for muscular dystrophy.

Report

(4 results)
  • 2014 Annual Research Report   Final Research Report ( PDF )
  • 2013 Annual Research Report
  • 2012 Annual Research Report
  • Research Products

    (25 results)

All 2015 2014 2013 2012 Other

All Journal Article (6 results) (of which Peer Reviewed: 6 results,  Open Access: 5 results) Presentation (17 results) Remarks (2 results)

  • [Journal Article] Fukutin is prerequisite to ameliorate muscular dystrophic phenotype by myofiber-selective LARGE expression.2015

    • Author(s)
      Ohtsuka Y, Kanagawa M, Yu CC, Ito C, Chiyo T, Kobayashi K, Okada T, Takeda S’I, Toda T.
    • Journal Title

      Sci Rep

      Volume: 5 Issue: 1 Pages: 8316-8316

    • DOI

      10.1038/srep08316

    • NAID

      120005600773

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Contribution of dysferlin deficiency to skeletal muscle pathology in asymptomatic and severe dystroglycanopathy models: generation of a new model for fukuyama congenital muscular dystrophy.2014

    • Author(s)
      Kanagawa M, Lu Z, Ito C, Matsuda C, Miyake K, Toda T.
    • Journal Title

      Plos ONE

      Volume: 9 Issue: 9 Pages: e106721-e106721

    • DOI

      10.1371/journal.pone.0106721

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] TRPV2 is critical for the maintenance of cardiac structure and function in mice.2014

    • Author(s)
      Katanosaka Y, Iwasaki K, Ujihara Y, Takatsu S, Nishitsuji K, Kanagawa M, Sudo A, Toda T, Katanosaka K, Mohri S, Naruse K.
    • Journal Title

      Nat Comm

      Volume: 5 Issue: 1 Pages: 3932-3932

    • DOI

      10.1038/ncomms4932

    • NAID

      120005690060

    • Related Report
      2014 Annual 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, 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
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Impaired viability of muscle precursor cells in muscular dystrophy with glycosylation defects and amelioration. of its severe phenotype by limited gene expression.2013

    • Author(s)
      Kanagawa M, et. al.
    • Journal Title

      Hum Mol Genet

      Volume: 22 Issue: 15 Pages: 3003-3015

    • DOI

      10.1093/hmg/ddt157

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed
  • [Journal Article] GTDC2 modifies O-mannosylated α-dystroglycan in the endoplasmic reticulum to generate N-acetyl-glucosamine epitopes reactive with CTD110.6 antibody.2013

    • Author(s)
      Ogawa M, Nakamura N, Nakayama Y, Kurosaka A, Manya H, Kanagawa M, Endo T, Furukawa K, Okajim T
    • Journal Title

      Biochem. Biophys. Res. Commun.

      Volume: 440 Issue: 1 Pages: 88-93

    • DOI

      10.1016/j.bbrc.2013.09.022

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed / Open Access
  • [Presentation] Pathophysiological roles for dystroglycan glycosylation in skeletal muscle and gene therapy challenge using glycosylation-deficient muscular dystrophy models2014

    • Author(s)
      Kanagawa, M., Yu, C-C., Fukada, S-I., Ohtsuka, Y., Ito, C., Chiyo, T., Okada,T., Takeda, S’I., Toda, T.
    • Organizer
      Society for Glycobiology (SFG) & Japanese Society of carbohydrate Research (JSCR) 2014 Joint Annual Meeting
    • Place of Presentation
      Honolulu
    • Year and Date
      2014-11-16 – 2014-11-19
    • Related Report
      2014 Annual Research Report
  • [Presentation] 糖鎖異常型筋ジストロフィーの病態と治療2014

    • Author(s)
      金川 基、戸田 達史
    • Organizer
      第87回日本生化学会大会
    • Place of Presentation
      京都
    • Year and Date
      2014-10-15 – 2014-10-18
    • Related Report
      2014 Annual Research Report
  • [Presentation] 糖鎖異常型筋ジストロフィーに対する遺伝子治療2014

    • Author(s)
      金川 基、大塚 喜久、游 智傑、伊藤 千代美、千代 智子、岡田 尚巳、武田 伸一、戸田 達史
    • Organizer
      第33回日本糖質学会
    • Place of Presentation
      名古屋
    • Year and Date
      2014-08-10 – 2014-08-12
    • Related Report
      2014 Annual Research Report
  • [Presentation] Impaired viability of muscle precursor cells in muscular dystrophy with glycosylation defects and amelioration of its severe phenotype by limited gene expression2014

    • Author(s)
      Kanagawa, M., Yu, C-C., Ito, C., Fukada, S-I., Chiyo, T., Kobayashia, K., Okada, T., Takeda, S’I. Toda, T.
    • Organizer
      13th International Congress on Neuromuscular Diseases
    • Place of Presentation
      Nice
    • Year and Date
      2014-07-05 – 2014-07-10
    • Related Report
      2014 Annual Research Report
  • [Presentation] Impaired viability of muscle precursor cells in muscular dystrophy with glycosylation defects and amelioration of its severe phenotype by limited gene expression2014

    • Author(s)
      Kanagawa, M., Yu, C-C., Ito, C., Fukada, S-I., Chiyo, T., Kobayashia, K., Okada, T., Takeda, S’I. Toda, T.
    • Organizer
      6th New Directions in Biology and Disease of Skeletal Muscle Conference
    • Place of Presentation
      Chicago
    • Year and Date
      2014-06-29 – 2014-07-02
    • Related Report
      2014 Annual Research Report
  • [Presentation] Impaired viability of muscle precursor cells in muscular dystrophy with glycosylation defects and amelioration of its severe phenotype by limited gene expression2013

    • Author(s)
      Kanagawa, M., Yu, C-C., Ito, C., Fukada, S-I., Chiyo, T., Kobayashia, K., Okada, T., Takeda, S’I. Toda, T.
    • Organizer
      18th International Congress of the World Muscle Society
    • Place of Presentation
      Asilomar, California, USA
    • Related Report
      2013 Annual Research Report
  • [Presentation] Impaired viability of muscle precursor cells in muscular dystrophy with glycosylation defects and amelioration of its severe phenotype by limited gene expression2013

    • Author(s)
      Kanagawa, M., Yu, C-C., Ito, C., Fukada, S-I., Chiyo, T., Kobayashia, K., Okada, T., Takeda, S’I. Toda, T.
    • Organizer
      EMBO Workshop
    • Place of Presentation
      Ascona, Switzerland
    • Related Report
      2013 Annual Research Report
  • [Presentation] 福山型筋ジストロフィーの発症機序と治療戦略2013

    • Author(s)
      金川 基、戸田 達史
    • Organizer
      第33回日本糖質学会
    • Place of Presentation
      大阪
    • Related Report
      2013 Annual Research Report
  • [Presentation] 糖鎖機能解析に基づく筋ジストロフィー病態の解明と治療法の開発2013

    • Author(s)
      金川 基
    • Organizer
      第33回日本糖質学会
    • Place of Presentation
      大阪
    • Related Report
      2013 Annual Research Report
  • [Presentation] 糖鎖機能と修飾機序の解明にもとづく筋ジストロフィー病態と治療法に関する研究2013

    • Author(s)
      金川 基
    • Organizer
      第86回日本生化学会大会
    • Place of Presentation
      横浜
    • Related Report
      2013 Annual Research Report
  • [Presentation] 2種類のフクチン欠損マウスを用いた福山型筋ジストロフィーの病態解析と遺伝子治療2013

    • Author(s)
      金川 基、游 智傑、伊藤 千代美、深田 宗一朗、千代 智子、小林 千浩、岡田 尚巳、武田 伸一、戸田 達史
    • Organizer
      第86回日本生化学会大会
    • Place of Presentation
      横浜
    • Related Report
      2013 Annual Research Report
  • [Presentation] 福山型筋ジストロフィーモデルマウスにおける筋前駆細胞の機能障害2013

    • Author(s)
      松尾 恵、金川 基、伊藤 千代美、小林 千浩、深田 宗一朗、戸田 達史
    • Organizer
      第36回日本分子生物学会年会
    • Place of Presentation
      神戸
    • Related Report
      2013 Annual Research Report
  • [Presentation] ジストログリカンのユニークな翻訳後修飾とその破綻による病態2013

    • Author(s)
      金川 基、戸田 達史
    • Organizer
      第36回日本分子生物学会年会
    • Place of Presentation
      神戸
    • Related Report
      2013 Annual Research Report
  • [Presentation] 糖鎖異常型筋ジストロフィーの発症機序と治療戦略2012

    • Author(s)
      金川基
    • Organizer
      第35回日本分子生物学会年会
    • Place of Presentation
      福岡(招待講演)
    • Year and Date
      2012-12-12
    • Related Report
      2012 Annual Research Report
  • [Presentation] ジストログリカンに見出された新規糖鎖修飾による機能制御と病態2012

    • Author(s)
      金川基
    • Organizer
      第31回日本糖質学会年会
    • Place of Presentation
      鹿児島
    • Year and Date
      2012-09-20
    • Related Report
      2012 Annual Research Report
  • [Presentation] Disruption of dystroglycan-pikachurin interaction underlies the molecular pathogenesis of eye abnormalities in dystroglycanopathy2012

    • Author(s)
      金川基
    • Organizer
      2012 New Directions in Biology and Disease of Skeletal Muscle Conference
    • Place of Presentation
      New Orleans (米国)
    • Year and Date
      2012-06-18
    • Related Report
      2012 Annual Research Report
  • [Presentation] ジストログリカンに見出された新規糖鎖修飾体の生理機能と筋ジストロフィー病態への関与2012

    • Author(s)
      金川基
    • Organizer
      第85回日本生化学会大会
    • Place of Presentation
      福岡
    • Related Report
      2012 Annual Research Report
  • [Remarks] 神戸大学大学院医学研究科 分子脳科学分野

    • URL

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

    • Related Report
      2013 Annual Research Report
  • [Remarks]

    • URL

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

    • Related Report
      2012 Annual Research Report

URL: 

Published: 2012-04-24   Modified: 2019-07-29  

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