@article{2f0baf86f2ef4398b3c04b1b24ccf3ad,
title = "Disruption of the sarcoglycan-sarcospan complex in vascular smooth muscle: A novel mechanism for cardiomyopathy and muscular dystrophy",
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 α-sarcoglycan (Sgca) or δ- sarcoglycan (Sgcd). We found that only Sgcd null mice developed cardiomyopathy with focal areas of necrosis as the histological hallmark in cardiac end 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 = "Ramon Coral-Vazquez and Cohn, {Ronald D.} and Moore, {Steven A.} and Hill, {Joseph A.} and Weiss, {Robert M.} and Davisson, {Robin L.} and Volker Straub and Rita Barresi and Dimple Bansal and Hrstka, {Ron F.} and Roger Williamson and Campbell, {Kevin P.}",
note = "Funding Information: We would like to thank all members of the Campbell laboratory, especially Rachelle H. Crosbie, Franck Duclos, Madeleine Durbeej, Michael D. Henry, Jane C. Lee, and David P. Venzke for the critical reading of the manuscript, fruitful discussions, and supply of critical reagents. We want to thank Kathryn Lamping for helpful comments on the pharmacological data. All DNA sequencing was carried out at the University of Iowa DNA Core facility (NIH DK25295). R. D. C. and V. S. were supported by the Deutsche Forschungsgemeinschaft. R. B. was supported by a grant from Telethon Italy N305/b. J. A. H. was supported by a grant from the Roy J. Carver Charitable Trust. R. M. W. was supported by a Grant-In-Aid from the American Heart Association. Histopathology slides were prepared in the Department of Pathology Research Lab. This work was also supported by the Muscular Dystrophy Association (R. W. and K. P. C.). K. P. C. is an Investigator of the Howard Hughes Medical Institute.",
year = "1999",
month = aug,
day = "20",
doi = "10.1016/S0092-8674(00)81975-3",
language = "English (US)",
volume = "98",
pages = "465--474",
journal = "Cell",
issn = "0092-8674",
publisher = "Cell Press",
number = "4",
}