Caveolin-1(-/-)- and caveolin-2(-/-)-deficient mice both display numerous skeletal muscle abnormalities, with tubular aggregate formation

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...

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Veröffentlicht in:	The American journal of pathology Jg. 170; H. 1; S. 316
Hauptverfasser:	Schubert, William, Sotgia, Federica, Cohen, Alex W, Capozza, Franco, Bonuccelli, Gloria, Bruno, Claudio, Minetti, Carlo, Bonilla, Eduardo, Dimauro, Salvatore, Lisanti, Michael P
Format:	Journal Article
Sprache:	Englisch
Veröffentlicht:	United States 01.01.2007
Schlagworte:	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
ISSN:	0002-9440
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Zusammenfassung:	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.
Bibliographie:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0002-9440
DOI:	10.2353/ajpath.2007.060687