Journal
ANNUAL REVIEW OF PHYSIOLOGY
Volume 71, Issue -, Pages 37-57Publisher
ANNUAL REVIEWS
DOI: 10.1146/annurev.physiol.010908.163216
Keywords
dystrophin; sarcoglycan; dysferlin; lamin A/C; nitric oxide synthase
Categories
Funding
- NIH [HL61322, ND47726]
- Muscular Dystrophy Association,
- Doris Duke Charitable Foundation
- Muscular Dystrophy Association
- [T32HL007381]
- [F32AR054700]
- NATIONAL HEART, LUNG, AND BLOOD INSTITUTE [R01HL061322, T32HL007381] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES [F32AR054700] Funding Source: NIH RePORTER
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To withstand the rigors of contraction, muscle fibers have specialized protein complexes that buffer against mechanical stress and a multi-faceted repair system that is rapidly activated after injury. Genetic studies first identified the mechanosensory signaling network that connects the structural elements of muscle and, more recently, have identified repair elements of muscle. Defects in the genes encoding the components of these systems lead to muscular dystrophy, a family of genetic disorders characterized by progressive muscle. wasting. Although the age of onset, affected muscles, and severity vary considerably, all muscular dystrophies are characterized by muscle necrosis that overtakes the regenerative capacity of muscle. The resulting replacement of muscle by fatty and fibrous tissue leaves muscle increasingly weak and nonfunctional. This review discusses the cellular mechanisms that are primarily and secondarily disrupted in muscular dystrophy, focusing on membrane degeneration, muscle regeneration, and the repair of muscle.
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