NDLI: Transgenic overexpression of caveolin-3 in skeletal muscle fibers induces a Duchenne-like muscular dystrophy phenotype.

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Transgenic overexpression of caveolin-3 in skeletal muscle fibers induces a Duchenne-like muscular dystrophy phenotype.

Content Provider	Semantic Scholar
Author	Galbiati, Ferruccio Volonté, Daniela Jeffrey Li, Mei Fine, Samson W. Fu, Mingui Bermúdez, J. Rafael Pedemonte, Marina Weidenheim, Karen M. Pestell, Richard G. Minetti, Carlo Lisanti, Michael Phillip
Copyright Year	2000
Abstract	It recently was reported that Duchenne muscular dystrophy (DMD) patients and mdx mice have elevated levels of caveolin-3 expression in their skeletal muscle. However, it remains unknown whether increased caveolin-3 levels in DMD patients contribute to the pathogenesis of DMD. Here, using a genetic approach, we test this hypothesis directly by overexpressing wild-type caveolin-3 as a transgene in mice. Analysis of skeletal muscle tissue from caveolin-3- overexpressing transgenic mice reveals: (i) a dramatic increase in the number of sarcolemmal muscle cell caveolae; (ii) a preponderance of hypertrophic, necrotic, and immature/regenerating skeletal muscle fibers with characteristic central nuclei; and (iii) down-regulation of dystrophin and beta-dystroglycan protein expression. In addition, these mice show elevated serum creatine kinase levels, consistent with the myo-necrosis observed morphologically. The Duchenne-like phenotype of caveolin-3 transgenic mice will provide an important mouse model for understanding the pathogenesis of DMD in humans.
Starting Page	397
Ending Page	406
Page Count	10
File Format	PDF HTM / HTML
Alternate Webpage(s)	http://www.pnas.org/content/97/17/9689.full.pdf
PubMed reference number	10931944v1
Volume Number	97
Issue Number	17
Journal	Proceedings of the National Academy of Sciences of the United States of America
Language	English
Access Restriction	Open
Subject Keyword	Caveolin 3 Cerebellar Nuclei Creatine Kinase DMD gene Dystroglycans Hypertrophy Kidney Papillary Necrosis MAJOR AFFECTIVE DISORDER 2 Muscle Cells Muscle Fibers Muscular Dystrophy Muscular Dystrophy, Duchenne Patients Skeletal Myocytes Skeletal muscle structure Tissue fiber Transgenes protein expression
Content Type	Text
Resource Type	Article

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