PMID:17200204

Citation	Schubert, W, Sotgia, F, Cohen, AW, Capozza, F, Bonuccelli, G, Bruno, C, Minetti, C, Bonilla, E, Dimauro, S and Lisanti, MP (2007) Caveolin-1(-/-)- and caveolin-2(-/-)-deficient mice both display numerous skeletal muscle abnormalities, with tubular aggregate formation. Am. J. Pathol. 170:316-33
Abstract	Here, we examine the role of "non-muscle" caveolins (Cav-1 and Cav-2) in skeletal muscle biology. Our results indicate that skeletal muscle fibers from male Cav-1(-/-) and Cav-2(-/-) mice show striking abnormalities, such as tubular aggregates, mitochondrial proliferation/aggregation, and increased numbers of M-cadherin-positive satellite cells. Notably, these skeletal muscle defects were more pronounced with increasing age. Because Cav-2-deficient mice displayed normal expression levels of Cav-1, whereas Cav-1-null mice exhibited an almost complete deficiency in Cav-2, these skeletal muscle abnormalities seem to be due to loss of Cav-2. Thus, Cav-2(-/-) mice represent a novel animal model-and the first genetically well-defined mouse model-that can be used to study the pathogenesis of tubular aggregate formation, which remains a poorly understood age-related skeletal muscle abnormality. Finally, because Cav-1 and Cav-2 were not expressed within mature skeletal myofibers, our results indicate that development of these abnormalities probably originates in stem/precursor cells, such as satellite cells or myoblasts. Consistent with this hypothesis, skeletal muscle isolated from male Cav-3(-/-) mice did not show any of these abnormalities. As such, this is the first study linking stem cells with the genesis of these intriguing muscle defects.
Links	PubMed PMC1762679 Online version:10.2353/ajpath.2007.060687
Keywords	Animals; Cadherins/biosynthesis; Caveolin 1/deficiency; Caveolin 1/genetics; Caveolin 2/deficiency; Caveolin 2/genetics; Disease Models, Animal; Electron Transport Complex IV/analysis; Genetic Predisposition to Disease; Male; Mice; Mice, Knockout; Microscopy, Electron, Transmission; Mitochondria, Muscle/metabolism; Mitochondria, Muscle/ultrastructure; Muscle Fibers, Skeletal/metabolism; Muscle Fibers, Skeletal/ultrastructure; Muscle, Skeletal/abnormalities; Muscle, Skeletal/metabolism; Muscle, Skeletal/ultrastructure; Muscular Diseases/genetics; Muscular Diseases/metabolism; Muscular Diseases/pathology; Myoblasts/metabolism; Myoblasts/pathology
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