Browse by author
Lookup NU author(s): Professor Volker StraubORCiD,
Dr Rita Barresi
Full text for this publication is not currently held within this repository. Alternative links are provided below where available.
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.
Author(s): Straub V; Barresi R; Coral-Vazquez R; Cohn RD; Moore SA; Hill JA; Weiss RM; Davisson RL; Bansal D; Hrstka RF; Williamson R; Campbell KP
Publication type: Article
Publication status: Published
Print publication date: 20/08/1999
ISSN (print): 0092-8674
ISSN (electronic): 1097-4172
Publisher: Cell Press
Altmetrics provided by Altmetric