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Abnormal function of aberrantly spliced ryanodine receptor and altered splicing in myotonic dystrophy brain.

Research Project

Project/Area Number 19591019
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionHyogo College of Medicine

Principal Investigator

YOSHIKAWA Hiroo  Hyogo College of Medicine, 医学部, 教授 (90273680)

Co-Investigator(Kenkyū-buntansha) KIMURA Takashi  兵庫医科大学, 医学部, 講師 (20441264)
Project Period (FY) 2007 – 2008
Project Status Completed (Fiscal Year 2008)
Budget Amount *help
¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2008: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2007: ¥3,770,000 (Direct Cost: ¥2,900,000、Indirect Cost: ¥870,000)
Keywords筋強直性ジストロフィー / スプライシング異常 / リアノジン受容体 / NMDA型グルタミン酸受容体 / Ca^<2+> / カルモジュリン依存性プロテインキナーゼII / 筋強直性ジストロフィー症 / スプライシング
Research Abstract

筋強直性ジストロフィー患者において、以前に我々が報告したリアノジン受容体スプライシング異常が、安静時、興奮時のCa^<2+>代謝に異常を生じさせることを新たに見出した。脳、心筋でのスプライシング異常を解析し、心筋ではリアノジン受容体タイプ2の新規スプライシング異常、脳ではリアノジン受容体タイプ1、NMDA型グルタミン酸受容体、およびCa^<2+>/カルモジュリン依存性プロテインキナーゼIIの新規スプライシング異常を見出した。

Report

(3 results)
  • 2008 Annual Research Report   Final Research Report ( PDF )
  • 2007 Annual Research Report
  • Research Products

    (25 results)

All 2009 2008 2007

All Journal Article (17 results) (of which Peer Reviewed: 16 results) Presentation (7 results) Book (1 results)

  • [Journal Article] Alternative splicing of RyR1 alters the efficacy of skeletal EC coupling.2009

    • Author(s)
      Kimura T, Lueck JD, Harvey PJ, Pace SM, Ikemoto N, Casarotto MG, Dirksen RT, Dulhunty AF.
    • Journal Title

      Cell Calcium 45

      Pages: 264-74

    • Related Report
      2008 Final Research Report
  • [Journal Article] Alternative splicing of RyRl alters the efficacy of skeletal EC coupling.2009

    • Author(s)
      Kimura T, Lueck JD, Harvey PJ, Pace SM, Ikemoto N, Casarotto MG, D irksen RT, Dulhunty AF
    • Journal Title

      Cell Calcium 45

      Pages: 264-74

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Phosphorylation of skeletal muscle calsequestrin enhances its Ca(2+) binding capacity and promotes its association with junctin.2008

    • Author(s)
      Beard NA, Wei L, Cheung SN, Kimura T, Varsanyi M, Dulhunty AF.
    • Journal Title

      Cell Calcium 44

      Pages: 363-73

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Muscle-specific GSTM2-2 on the luminal side of the sarcoplasmic reticulum modifies RyR ion channel activity.2008

    • Author(s)
      Wei L, Abdellatif YA, Liu D, Kimura T, 以下5名省略
    • Journal Title

      Int J Biochem Cell Biol. 40

      Pages: 1616-28

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Aberrantly spliced alpha-dystrobrevin alters alpha-syntrophin binding in myotonic dystrophy type 1.2008

    • Author(s)
      Nakamori M, Kimura T, 以下6名省略
    • Journal Title

      Neurology 70

      Pages: 677-85

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Circulating CD34-positive cells provide a marker of vascular risk associated with cognitive impairment.2008

    • Author(s)
      Taguchi A, Matsuyama T, Nakagomi T, Shimizu Y, Fukunaga R, Tatsumi Y, Yoshikawa H, 以下6名省略
    • Journal Title

      Journal of Cerebral Blood Flow & Metabolism 28

      Pages: 445-449

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Increasing in circulating CD34-positive cells in patients with angiographic evidence of moyamoya-like vessels.2008

    • Author(s)
      Yoshihara T, Taguchi A, Matsuyama T, Shimizu Y, Kikuchi-Taura A, Soma T, Stern DM , Yoshikawa H,以下4名省略
    • Journal Title

      Journal of Cerebral Blood Flow & Metabolism. 28

      Pages: 1086-89

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Mice lacking the schizophrenia-associated protein FEZ-1 manifest hyperactivity and enhanced responsiveness to psychostimulants.2008

    • Author(s)
      Sakae N, Yamasaki N, Tsukada T, Yamada M, Yoshikawa H, 以下6名省略
    • Journal Title

      Hum Mol Genet. 17

      Pages: 3191-203

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Pioglitazone promotes peripheral nerve remyelination after crush injury through CD36 upregulation.2008

    • Author(s)
      Eto M, Sumi H, Fujimura H, Yoshikawa H, Sakoda S,
    • Journal Title

      J Peripher Nerv Syst. 13

      Pages: 242-8

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Phosphorylation of skeletal muscle calsequestrin enhances its Ca2+binding capacity and promotes its association with junctin.2008

    • Author(s)
      Beard NA, Wei L, Cheung SN, Kimura T, Varsanyi M, Dulhunty AF
    • Journal Title

      Cell Calcium 44

      Pages: 363-73

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] A variably spliced region in the type 1 ryanodine receptor may participate in an inter-domain interaction.2007

    • Author(s)
      Kimura T, Pace SM, Wei L, Beard NA, Dirksen RT, Dulhunty AF.
    • Journal Title

      Biochem J. 401

      Pages: 317-24

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Triadin binding to the C-terminal luminal loop of the ryanodine receptor is important for skeletal muscle excitation contraction coupling.2007

    • Author(s)
      Goonasekera SA, Beard NA, Groom L, Kimura T, 以下5名省略
    • Journal Title

      J Gen Physiol. 130

      Pages: 365-78

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Altered mRNA splicing of dystrophin in type 1 myotonic dystrophy.2007

    • Author(s)
      Nakamori M, Kimura T, Fujimura H, Takahashi MP, Sakoda S.
    • Journal Title

      Muscle Nerve. 36

      Pages: 251-7

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Endoplasmic reticulum stress in myotonic dystrophy type 1 muscle2007

    • Author(s)
      Ikezoe K, Nakamori M, Furuya H, Arahata H, Kanemoto S, Kimura T, 4名省略, Kira J.
    • Journal Title

      Acta Neuropathol. 114

      Pages: 527-35

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Nonmyelinating Schwann cell involvement with well preserved unmyelinated axons inCMT1A.2007

    • Author(s)
      Koike H, Iijima M, Mori K, Yamamoto M, Hattori N, Katsuno M, Tanaka F, Watanabe H, Doyu M, Yoshikawa H,, Sobue G.
    • Journal Title

      J Neuropathol Exp Neurol. 66

      Pages: 1027-1036

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] Recognition memory for unfamiliar faces in Parkinson's disease: Behavioral and electrophysiologic measures.2007

    • Author(s)
      Kida Y, Tachibana H, Takeda M, Yoshikawa H, Okita T.
    • Journal Title

      Parkinsonism and Related disorders. 13

      Pages: 157-164

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Journal Article] inad マウスにおける末梢神経病変2007

    • Author(s)
      辰巳美晶、辰己由記、芳川浩男
    • Journal Title

      末梢神経 Peripheral Nerve. 18

      Pages: 53-60

    • NAID

      10027577928

    • Related Report
      2008 Final Research Report
    • Peer Reviewed
  • [Presentation] 筋強直性ジストロフィー症におけるα-dystrobrevin スプライシング異常2008

    • Author(s)
      高橋正紀, 中森雅之, 木村卓, 久保田智哉, 松村剛, 隅寿恵, 藤村晴俊, 佐古田三郎
    • Organizer
      第49回日本神経学会総会
    • Place of Presentation
      横浜
    • Related Report
      2008 Final Research Report
  • [Presentation] 筋強直性ジストロフィー脳、心筋におけるスプライシング異常の検討2008

    • Author(s)
      木村卓, 中森雅之, 高橋正紀, 松村剛, 藤村晴俊, 陣内研二, 芳川浩男
    • Organizer
      第49回日本神経学会総会
    • Place of Presentation
      横浜
    • Related Report
      2008 Annual Research Report 2008 Final Research Report
  • [Presentation] 筋強直性ジストロフィー症におけるα-dystrobrevinスプライシング異常2008

    • Author(s)
      高橋正紀(大阪大学神経内科), 中森雅之, 木村卓, 久保田智哉, 松村剛, 隅寿恵, 藤村晴俊, 佐古田三郎
    • Organizer
      第49回日本神経学会総会
    • Place of Presentation
      横浜
    • Related Report
      2008 Annual Research Report
  • [Presentation] A variably spliced region of Ryanodine receptor 1 may be involved in excitation-contraction coupling.2007

    • Author(s)
      Kimura, T., Nakamori, M., Takahashi, M. P., Yoshikawa, H., Sakoda, S., and Dulhunty, A. F.
    • Organizer
      6^<th> International Myotonic Dystrophy Consortium Meeting
    • Place of Presentation
      イタリア、ミラノ
    • Related Report
      2008 Final Research Report
  • [Presentation] 筋強直性ジストロフィー症にみられるリアノジン受容体機能異常のメカニズム2007

    • Author(s)
      木村卓, 中森雅之, 高橋正紀, Dulhunty Angela F., 芳川浩男, 佐古田三郎
    • Organizer
      第48回日本神経学会総会
    • Related Report
      2008 Final Research Report
  • [Presentation] 筋強直性ジストロフィー症にみられるリアノジン受容体機能異常のメカニズム2007

    • Author(s)
      木村 卓, 中森 雅之, 高橋 正紀, Dulhunty Angela F., 芳川 浩男, 佐古田 三郎
    • Organizer
      第48回日本神経学会総会
    • Place of Presentation
      名古屋
    • Related Report
      2007 Annual Research Report
  • [Presentation] A variably spliced region of Ryanodine receptor 1 may be involved in excitation-contraction coupling2007

    • Author(s)
      Kimura, T., Nakamori, M., Takahashi, M. P., Yoshikawa, H., Sakoda, S., Dulhunty, A. F.
    • Organizer
      6th International Myotonic Dystrophy Consortium Meeting
    • Place of Presentation
      イタリア、ミラノ
    • Related Report
      2007 Annual Research Report
  • [Book] 【処方計画法】神経・筋疾患低K性周期性四肢麻痺(解説/特集)2008

    • Author(s)
      木村卓
    • Total Pages
      688
    • Publisher
      永井書店
    • Related Report
      2008 Annual Research Report

URL: 

Published: 2007-04-01   Modified: 2016-04-21  

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