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

Ramon Coral-Vazquez, Ronald D. Cohn, Steven A. Moore, Joseph A. Hill, Robert M. Weiss, Robin L. Davisson, Volker Straub, Rita Barresi, Dimple Bansal, Ron F. Hrstka, Roger Williamson, Kevin P. Campbell

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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 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.
Original languageAmerican English
Pages (from-to)465-474
Number of pages10
JournalCell
DOIs
StatePublished - 20 Aug 1999
Externally publishedYes

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Sarcoglycans
Muscular Dystrophies
Cardiomyopathies
Vascular Smooth Muscle
Muscle
Necrosis
Dystrophin
Blood Vessels
Glycoproteins
Animals
Skeletal Muscle
Animal Models
Membranes
Mutation

Cite this

Coral-Vazquez, Ramon ; Cohn, Ronald D. ; Moore, Steven A. ; Hill, Joseph A. ; Weiss, Robert M. ; Davisson, Robin L. ; Straub, Volker ; Barresi, Rita ; Bansal, Dimple ; Hrstka, Ron F. ; Williamson, Roger ; Campbell, Kevin P. / Disruption of the sarcoglycan-sarcospan complex in vascular smooth muscle: A novel mechanism for cardiomyopathy and muscular dystrophy. In: Cell. 1999 ; pp. 465-474.
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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.}",
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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 1999, 'Disruption of the sarcoglycan-sarcospan complex in vascular smooth muscle: A novel mechanism for cardiomyopathy and muscular dystrophy', Cell, pp. 465-474. https://doi.org/10.1016/S0092-8674(00)81975-3

Disruption of the sarcoglycan-sarcospan complex in vascular smooth muscle: A novel mechanism for cardiomyopathy and muscular dystrophy. / Coral-Vazquez, Ramon; Cohn, Ronald D.; Moore, Steven A.; Hill, Joseph A.; Weiss, Robert M.; Davisson, Robin L.; Straub, Volker; Barresi, Rita; Bansal, Dimple; Hrstka, Ron F.; Williamson, Roger; Campbell, Kevin P.

In: Cell, 20.08.1999, p. 465-474.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Disruption of the sarcoglycan-sarcospan complex in vascular smooth muscle: A novel mechanism for cardiomyopathy and muscular dystrophy

AU - Coral-Vazquez, Ramon

AU - Cohn, Ronald D.

AU - Moore, Steven A.

AU - Hill, Joseph A.

AU - Weiss, Robert M.

AU - Davisson, Robin L.

AU - Straub, Volker

AU - Barresi, Rita

AU - Bansal, Dimple

AU - Hrstka, Ron F.

AU - Williamson, Roger

AU - Campbell, Kevin P.

PY - 1999/8/20

Y1 - 1999/8/20

N2 - 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.

AB - 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.

U2 - 10.1016/S0092-8674(00)81975-3

DO - 10.1016/S0092-8674(00)81975-3

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