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

Lookup NU author(s): Professor Volker Straub, Dr Rita Barresi

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Abstract

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.


Publication metadata

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

Journal: Cell

Year: 1999

Volume: 98

Issue: 4

Pages: 465-474

Print publication date: 20/08/1999

ISSN (print): 0092-8674

ISSN (electronic): 1097-4172

Publisher: Cell Press

URL: http://dx.doi.org/10.1016/S0092-8674(00)81975-3

DOI: 10.1016/S0092-8674(00)81975-3


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