Title |
Dystrophin deficiency exacerbates skeletal muscle pathology in dysferlin-null mice
|
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Published in |
Skeletal Muscle, December 2011
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DOI | 10.1186/2044-5040-1-35 |
Pubmed ID | |
Authors |
Renzhi Han, Erik P Rader, Jennifer R Levy, Dimple Bansal, Kevin P Campbell |
Abstract |
Mutations in the genes coding for either dystrophin or dysferlin cause distinct forms of muscular dystrophy. Dystrophin links the cytoskeleton to the sarcolemma through direct interaction with β-dystroglycan. This link extends to the extracellular matrix by β-dystroglycan's interaction with α-dystroglycan, which binds extracellular matrix proteins, including laminin α2, agrin and perlecan, that possess laminin globular domains. The absence of dystrophin disrupts this link, leading to compromised muscle sarcolemmal integrity. Dysferlin, on the other hand, plays an important role in the Ca2+-dependent membrane repair of damaged sarcolemma in skeletal muscle. Because dysferlin and dystrophin play different roles in maintaining muscle cell integrity, we hypothesized that disrupting sarcolemmal integrity with dystrophin deficiency would exacerbate the pathology in dysferlin-null mice and allow further characterization of the role of dysferlin in skeletal muscle. |
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