ePrints

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

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

Downloads

Full text for this publication is not currently held within this repository. Alternative links are provided below where available.

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

Altmetrics

Share