Journal article
Disruption of the Sarcoglycan–Sarcospan Complex in Vascular Smooth Muscle: A Novel Mechanism for Cardiomyopathy and Muscular Dystrophy
Cell (Cambridge), Vol.98(4), pp.465-474
1999
DOI: 10.1016/S0092-8674(00)81975-3
PMID: 10481911
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 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.
Details
- Title: Subtitle
- Disruption of the Sarcoglycan–Sarcospan Complex in Vascular Smooth Muscle: A Novel Mechanism for Cardiomyopathy and Muscular Dystrophy
- Creators
- Ramon Coral-Vazquez - Department of Physiology and Biophysics, and Department of Neurology, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USARonald D Cohn - Department of Physiology and Biophysics, and Department of Neurology, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USASteven A Moore - Department of Pathology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USAJoseph A Hill - Department of Veterans Affairs and Department of Internal Medicine, University of Iowa, Iowa City, Iowa 52242, USARobert M Weiss - Department of Veterans Affairs and Department of Internal Medicine, University of Iowa, Iowa City, Iowa 52242, USARobin L Davisson - Department of Anatomy and Cell Biology, University of Iowa, Iowa City, Iowa 52242, USAVolker Straub - Department of Physiology and Biophysics, and Department of Neurology, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USARita Barresi - Department of Physiology and Biophysics, and Department of Neurology, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USADimple Bansal - Department of Physiology and Biophysics, and Department of Neurology, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USARon F Hrstka - Department of Obstetrics and Gynecology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USARoger Williamson - Department of Obstetrics and Gynecology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USAKevin P Campbell - Department of Physiology and Biophysics, and Department of Neurology, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
- Resource Type
- Journal article
- Publication Details
- Cell (Cambridge), Vol.98(4), pp.465-474
- Publisher
- Elsevier Inc
- DOI
- 10.1016/S0092-8674(00)81975-3
- PMID
- 10481911
- ISSN
- 0092-8674
- eISSN
- 1097-4172
- Language
- English
- Date published
- 1999
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Pathology; Iowa Neuroscience Institute; Cardiovascular Medicine; Obstetrics and Gynecology; Internal Medicine
- Record Identifier
- 9984047993302771
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