Dystrophic-dwarf mice: Pathogenesis of muscular dystrophy
| Project/Area Number |
61571086
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
医学一般
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| Research Institution | Institute for Developmental Research, Aichi Human Service Center |
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Principal Investigator |
TOTSUKA Tsuyoshi Institute for Developmental Research, Aichi Prefectural Colony, 生理学部門, 研究員 (20100167)
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| Project Period (FY) |
1986 – 1988
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| Project Status |
Completed (Fiscal Year 1988)
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| 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)
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| Keywords | Muscular 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 |
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| 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 (
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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
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Report
(4 results)
Research Products
(26 results)
