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FORMATION PROCESS OF DYSTROPHIN-POSITIVE MUSCLE FIBERS FOLLOWING THE MYOBLAST TRANSPLANTATION TO MDX MICE.

Research Project

Project/Area Number 05670215
Research Category

Grant-in-Aid for General Scientific Research (C)

Allocation TypeSingle-year Grants
Research Field Experimental pathology
Research InstitutionNATIONAL INSTITUTE OF NEUROSCIENCE,NCNP

Principal Investigator

HAGIWARA Yasuko  NATIONAL INSTITUTE OF NUROSCIENCE,NCNP SECTION CHIEF, 神経研究所, 室長 (00175530)

Co-Investigator(Kenkyū-buntansha) MIZUNO Yuji  NATIONAL INSTITUTE OF NUROSCIENCE,NCNP POST DOCTOR FELLOW, 神経センター・神経研究所, 併任研究員
Project Period (FY) 1993 – 1994
Project Status Completed (Fiscal Year 1994)
Budget Amount *help
¥2,000,000 (Direct Cost: ¥2,000,000)
Fiscal Year 1994: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 1993: ¥1,200,000 (Direct Cost: ¥1,200,000)
KeywordsMuscular dystrophy / Skeletal muscle / Myoblast transplantation / Dystrophin / mdx mouse / C2 cell / 遺伝子導入法 / カチオン性脂質法
Research Abstract

Dystrophin is a responsible protein of Duchenne muscular dystrophy(DMD) and is present at the protoplasmic surface of normal sarcolemma. Its absence is observed in DMD and mdx (mouse equivalent to DMD) muscles and is considered to be the cause of muscle fiber degeneration. To construct the subsarcolemmal undercoat in mdx muscle for the trial of therapy of the disease, myoblasts with normal dystrophy gene are injected into mdx mouse muscle. In this study we intended to elucidate the mechanism how dystrophin-positive fibers are constructed following myoblast transfer.
We injected C2 myoblasts, a normal mouse muscle cell line, into the muscles of mdx nude mouse. This resulted in wide-spread necrosis of the injected muscle which was followed by regeneration. A large number of dystrophin-positive fibers appeared at the regeneration area. Isoformes of glucose-6-phosphate isomerase homodimer were used for the analysis, which are different in C2 and mdx cells. In dystrophin-positive muscle, a hybrid enzyme was observed, suggesting many of each fiber contain both normal and mdx muclei. Such hybrid fibers may not be resulted from uptake of C2 myoblasts to the intact mdx fibers, but from fusion of C2 myoblasts and mdx satellite cell generated on necrosis of the muscle fibers, considering the fiber formation process and the sizes of these fibers. In addition to this, the presence of muscle fibers made from fusion of C2 myoblasts cannot be excluded.

Report

(3 results)
  • 1994 Annual Research Report   Final Research Report Summary
  • 1993 Annual Research Report
  • Research Products

    (13 results)

All Other

All Publications (13 results)

  • [Publications] 萩原康子: "A new method for fibroblast-less primary skeletal muscle cell culture by the use of hydroxyurea." Develop.Growth.Differ.36. 141-148 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 水野裕司: "Expression of utrophin(dystrophin-related protein)and dystrophin-associated glycoproteins in muscles from patients with duchenne muscular dystrophy." Muscle Nerve. 17. 206-216 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 水野裕司: "Selective defect of sarcoglycan complex in severe selective childhood autosomal recessive muscular dystrophy muscle." Biochem.Biophys.Res.Comm.203. 979-983 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 水野裕司: "Sarcoglycan complex is selectively lost in dystrophic hamster muscle." Am.J.Pathology. 146. 530-536 (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 萩原康子: "A new method for fibroblast-less primary skeletal muscle cell culture by the use of hydroxyurea." Develop.Growth.Differ.36. 141-148 (1994)

    • Related Report
      1994 Annual Research Report
  • [Publications] 水野裕司: "Expression of utrophin(dystrophin-related protein)and dystrophin-associated glycoproteins in muscles from patients with duchenne muscular dystrophy." Muscle Nerve. 17. 206-216 (1994)

    • Related Report
      1994 Annual Research Report
  • [Publications] 水野裕司: "Selective defect of sarcoglycan complex in severe selective childhood autosomal recessive muscular dystrophy muscle." Biochem.Biophys.Res.Comm.203. 979-983 (1994)

    • Related Report
      1994 Annual Research Report
  • [Publications] 水野裕司: "Sarcoglycan complex is selectively lost in dystrophic hamster muscle." Am.J.Pathology. 146. 530-536 (1995)

    • Related Report
      1994 Annual Research Report
  • [Publications] 萩原康子: "Modes of appearance of dystrophin‐positive muscle fibers following myoblast transfer into mdx nude mice" Cell Struct Funct. (発表予定)18. (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] 萩原康子: "Formation of dystrophin‐positive muscle fibers in the mdx mouse by C2 myoblast transfer" Jpn J Pharmacol. (発表予定)64. (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] 萩原,康子: "A new method for fibroblast‐less primary skeletal muscle cell culture by the use of hydroxyurea" Dev Growth Differ. (発表予定)36. (1994)

    • Related Report
      1993 Annual Research Report
  • [Publications] 水野裕司: "Reciprocal expression of dystophin and utrophin in muscles of Duchenne muscular dystrophy patients,female DMD‐carriers and control subjects" J Neurol Sci. 119. 43-52 (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] 水野裕司: "Expression of utrophin(dystrophin related protein)and dystrophin‐associated glycoproteins in muscles from patients" Muscle Nerve. 17. 206-216 (1994)

    • Related Report
      1993 Annual Research Report

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Published: 1993-04-01   Modified: 2016-04-21  

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