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Dystrophic-dwarf mice: Pathogenesis of muscular dystrophy

Research Project

Project/Area Number 61571086
Research Category

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

Allocation TypeSingle-year Grants
Research Field 医学一般
Research InstitutionInstitute for Developmental Research, Aichi Human Service Center

Principal Investigator

TOTSUKA Tsuyoshi  Institute for Developmental Research, Aichi Prefectural Colony, 生理学部門, 研究員 (20100167)

Project Period (FY) 1986 – 1988
Project Status Completed (Fiscal Year 1988)
Budget Amount *help
¥2,300,000 (Direct Cost: ¥2,300,000)
Fiscal Year 1988: ¥700,000 (Direct Cost: ¥700,000)
Fiscal Year 1987: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 1986: ¥800,000 (Direct Cost: ¥800,000)
KeywordsMuscular Dystrophy / Dwarf / Mouse / Defective Maturation / Muscle / Bone / Esophagus / 舌 / 筋-骨 / 対症療法 / 重複疾患 / 筋一骨 / 中心核
Research Abstract

Since our proposal of a bone-muscle imbalance hypothesis for the pathogenesis of mouse muscular dystrophy (dy), many facts have been found in support of this hypothesis: subsequent reports by other authors). 2. A growth inhibition or a small stature has a beneficial effect (King et al. on dy chickens; Totsuka et al. on dy-dwarf mice; Zatz et al. on Duchenne dy patients; Karpati et al. on dy hamsters; Valentine et al. on dy dogs). 3. There is no correlation between the degrees of severity in pathological changes and functional disabilities of muscles (Totsuka et al. and Komatsu et al. on foreleg muscles of dy mice; Totsuka et al. on dy-dwarf mice). 4. Immobilization of muscles has a beneficial effect (Wirtz et al.); functional overload has a damaging effect (Vrbova et al.) on muscles in dy mice. 5. The sarcomere of dy chichen muscle at the resting state is abnormally extended (Ashmore et al.). 6. Passive stretch of adult chicken muscle produces a myopathy similar to muscular dystrophy ( … More Ashmore et al.). 7. The present studies have demonstrated that tongue and esophagus muscle fibers which are uninfluenced by the bone growth are free of pathological changes in dy mice.
Morphometrical and histochemical studies on dy mouse muscles have shown that pathological changes (a fiber-size variation, central nucleation and fiber splitting) are not the result of degeneration and impaired regeneration but a reflex of defective maturation of muscle fibers and its secondary phenomena caused by an age-related aggravation in bone-muscle imbalance.
Despite recent vigorous progress in research on dystrophin which is assumed to be the protein responsible for Duchenne muscular dystrophy, the etiology of the disease is unknown. Some authors have postulated that dystrophin might be a cytoskeletal protein giving strength to surface membrane. This is consistent in part with our bone-muscle imbalance hypothesis. Further studies along multifarious lines of thinking are still necessary to reveal the pathogenesis of muscular dystrophies. Less

Report

(4 results)
  • 1988 Annual Research Report   Final Research Report Summary
  • 1987 Annual Research Report
  • 1986 Annual Research Report
  • Research Products

    (26 results)

All Other

All Publications (26 results)

  • [Publications] T.Totsuka: Congen.Anomal.26. 157-167 (1986)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] T.Totsuka: J.Physiol.Soc.Japan. 48. 703-706 (1986)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] T.Totsuka: Congen.Anomal.27. 51-60 (1987)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] T.Totsuka: Proc.Japan Acad.63B. 131-134 (1987)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] T.Totsuka.;I.Uramoto: J.Physiol.Soc.Japan. 50. 533 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] T.Totsuka: Neurosci.Res.Suppl.7. S63 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] T. Totsuka: "A paradoxical growth of large myofibers in growth-arrested rectus femoris muscles of muscular dystrophic mice" Congen. Anomal.26. 157-167 (1986)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] T. Totsuka: "A variation in growth capability of myofibers shifted to incompetence as the pathogenesis of mouse muscular dystrophy" J. Physiol. Soc. Japan. 48. 703-706 (1986)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] T. Totsuka: "Centronucleated myofibers having also peripheral nuclei in rectus femoris muscles of muscular dystrophic mice" Congen. Anomal.27. 51-60 (1987)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] T. Totsuka: "Normal diameter distribution of tongue muscle fibers in muscular dystrophic mice" Proc. Japan Acad.63B. 131-134 (1987)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] T. Totsuka, I. Uramoto: "Muscular dystrophic mice: Centronucleated muscle fibers are otherwise within the other fibers" J. Physiol. Soc. Japan. 50. 533 (1988)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] T.Totsuka,;I.Uramoto: J.Physiol.Soc.Japan. 50. 533 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] T.Totsuka: Neurosci.Res.Suppl.7. S63 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] 戸塚武、渡辺貴美、長浜眞人: 愛知県発達障害研究所年報. (1989)

    • Related Report
      1988 Annual Research Report
  • [Publications] 渡辺貴美、戸塚武: 愛知県発達障害研究所年報. (1989)

    • Related Report
      1988 Annual Research Report
  • [Publications] 日高輝明、丸山直亮、水谷隆治、戸塚武: 愛知県発達障害研究所年報. (1989)

    • Related Report
      1988 Annual Research Report
  • [Publications] K.Watanabe,;I.Uramoto,;T.Totsuka: Neurosci.Res.(1989)

    • Related Report
      1988 Annual Research Report
  • [Publications] T. Totsuka: Proc. Japan Acad.63B. 131-134 (1987)

    • Related Report
      1987 Annual Research Report
  • [Publications] T. Totsuka: Neuroscience Research. (1988)

    • Related Report
      1987 Annual Research Report
  • [Publications] 戸塚 武: 愛知県発達障害研究所年報. 16. (1988)

    • Related Report
      1987 Annual Research Report
  • [Publications] 日高輝明,水谷隆治,戸塚武: 愛知県発達障害研究所年報. 16. (1988)

    • Related Report
      1987 Annual Research Report
  • [Publications] 布藤智康,日高輝明,水谷隆治,戸塚武,奥山治美: 愛知県発達障害研究所年報. 16. (1988)

    • Related Report
      1987 Annual Research Report
  • [Publications] K.Kato, A. Shimizu, T.Totsuka: J. Neurol.Sci.(1988)

    • Related Report
      1987 Annual Research Report
  • [Publications] T.Totsuka: Congenital Anomalies. 26. 157-167 (1986)

    • Related Report
      1986 Annual Research Report
  • [Publications] T.Totsuka: J.Physiological Society of Japan. 48. 703-706 (1986)

    • Related Report
      1986 Annual Research Report
  • [Publications] T.Totsuka: Congenital Anomalies. 27. (1987)

    • Related Report
      1986 Annual Research Report

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Published: 1987-03-31   Modified: 2016-04-21  

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