Functional characteristics of dystrophic skeletal muscle: insights from animal models

1  Department of Pediatrics, Magee-Women's Research Institute, Duchenne Muscular Dystrophy Research Center, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15213; and 2  Research Center for Genetic Medicine, Children's National Medical Center, Duchenne Muscular Dystro...

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Bibliographic Details
Published in:Journal of applied physiology (1985) 2002-08, Vol.93 (2), p.407-417
Main Authors: Watchko, Jon F, O'Day, Terrence L, Hoffman, Eric P
Format: Article
Language:English
Subjects:
Anatomy & physiology
Animals
Biological and medical sciences
Disease Models, Animal
Diseases of striated muscles. Neuromuscular diseases
Humans
Medical sciences
Mice
Mice, Inbred mdx
Muscle, Skeletal - physiopathology
Muscular dystrophy
Muscular Dystrophy, Animal - genetics
Muscular Dystrophy, Animal - physiopathology
Muscular Dystrophy, Duchenne - genetics
Muscular Dystrophy, Duchenne - physiopathology
Neurology
Skeletal system
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Summary:1  Department of Pediatrics, Magee-Women's Research Institute, Duchenne Muscular Dystrophy Research Center, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15213; and 2  Research Center for Genetic Medicine, Children's National Medical Center, Duchenne Muscular Dystrophy Research Center, Washington, DC 20010 Muscular dystrophies are a clinically and genetically heterogeneous group of disorders that show myofiber degeneration and regeneration. Identification of animal models of muscular dystrophy has been instrumental in research on the pathogenesis, pathophysiology, and treatment of these disorders. We review our understanding of the functional status of dystrophic skeletal muscle from selected animal models with a focus on 1 ) the mdx mouse model of Duchenne muscular dystrophy, 2 ) the Bio 14.6  -sarcoglycan-deficient hamster model of limb-girdle muscular dystrophy, and 3 ) transgenic null mutant murine lines of sarcoglycan ( , , , and ) deficiencies. Although biochemical data from these models suggest that the dystrophin-sarcoglycan-dystroglycan-laminin network is critical for structural integrity of the myofiber plasma membrane, emerging studies of muscle physiology suggest a more complex picture, with specific functional deficits varying considerably from muscle to muscle and model to model. It is likely that changes in muscle structure and function, downstream of the specific, primary biochemical deficiency, may alter muscle contractile properties. dystrophin; mdx mouse; sarcoglycanopathies
ISSN:8750-7587
1522-1601
DOI:10.1152/japplphysiol.01242.2001