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

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


  • Coral-Vazquez R
  • Cohn RD
  • Moore SA
  • Hill JA
  • Weiss RM
  • Davisson RL
  • Straub V
  • Barresi R
  • Bansal D
  • Hrstka RF
  • Williamson R
  • Campbell KP



Publication Data

August 20, 1999

Associated Grants

  • Agency: NIDDK NIH HHS, Id: DK25295

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