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

Research output: Contribution to journalArticle

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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 languageEnglish (US)
Pages (from-to)465-474
Number of pages10
JournalCell
Volume98
Issue number4
DOIs
StatePublished - Aug 20 1999

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

ASJC Scopus subject areas

  • Cell Biology
  • Molecular Biology

Cite this

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, Vol. 98, No. 4, 20.08.1999, p. 465-474.

Research output: Contribution to journalArticle

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, vol. 98, no. 4, pp. 465-474. https://doi.org/10.1016/S0092-8674(00)81975-3
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 ; Vol. 98, No. 4. pp. 465-474.
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