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Disruption of the sarcoglycan-sarcospan complex in vascular smooth muscle: a novel mechanism for cardiomyopathy and muscular dystrophy.

Cell | Aug 20, 1999

http://www.ncbi.nlm.nih.gov/pubmed/10481911

To investigate mechanisms in the pathogenesis of cardiomyopathy associated with mutations of the dystrophin-glycoprotein complex, we analyzed genetically engineered mice deficient for either alpha-sarcoglycan (Sgca) or delta-sarcoglycan (Sgcd). We found that only Sgcd null mice developed cardiomyopathy with focal areas of necrosis as the histological hallmark in cardiac and skeletal muscle. Absence of the sarcoglycan-sarcospan (SG-SSPN) complex in skeletal and cardiac membranes was observed in both animal models. Loss of vascular smooth muscle SG-SSPN complex was only detected in Sgcd null mice and associated with irregularities of the coronary vasculature. Administration of a vascular smooth muscle relaxant prevented onset of myocardial necrosis. Our data indicate that disruption of the SG-SSPN complex in vascular smooth muscle perturbs vascular function, which initiates cardiomyopathy and exacerbates muscular dystrophy.

Pubmed ID: 10481911 RIS Download

Mesh terms: Animals | Cardiomyopathy, Dilated | Carrier Proteins | Coronary Vessels | Cytoskeletal Proteins | Macromolecular Substances | Membrane Glycoproteins | Membrane Proteins | Mice | Mice, Knockout | Muscle, Smooth, Vascular | Muscular Dystrophy, Animal | Myocardium | Necrosis | Neoplasm Proteins | Physical Conditioning, Animal | Sarcoglycans

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Associated grants

  • Agency: NIDDK NIH HHS, Id: DK25295

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