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First published online 13 July 2004
doi: 10.1242/jcs.01234
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Research Article |
7ß1 in muscle
1 Department of Medicine, The University of Chicago, 5841 South Maryland Avenue, Chicago, IL 60637, USA
2 Department of Human Genetics, The University of Chicago, 5841 South Maryland Avenue, Chicago, IL 60637, USA
3 Wellcome Trust Centre for Cell-Matrix Research, School of Biological Sciences, University of Manchester, 2.205 Stopford Building, Oxford Road, Manchester, M13 9PT, UK
* Author for correspondence (e-mail: emcnally{at}medicine.bsd.uchicago.edu)
Accepted 23 March 2004
Disruption of the sarcoglycan complex leads to muscle membrane instability and muscular dystrophy in humans and mice. Through the dystrophin glycoprotein complex, sarcoglycan participates in connecting the internal cytoskeleton to the membrane and the extracellular matrix. Integrin 
7ß1 is also a transmembrane protein of skeletal and cardiac muscle that similarly links the cytoskeleton to the extracellular matrix. Mice lacking integrin 7 develop mild muscle degeneration, while sarcoglycan mutant mice display overt muscle degeneration and muscular dystrophy. In sarcoglycan-deficient muscle, integrin 7 protein was upregulated at the plasma membrane. To ascertain whether integrin 7 upregulation compensates for the loss of the transmembrane sarcoglycan linkage in sarcoglycan-deficient muscle, we generated mice lacking both integrin 7 and 
-sarcoglycan (gxi). These double-mutant gxi mice exhibit profound, rapid muscle degeneration leading to death before one month of age consistent with a weakened cellular attachment to the extracellular matrix. The regenerative capacity of gxi muscle was intact with increased embryonic myosin heavy chain expression, myofiber central nucleation and normal in vivo myoblast differentiation. Therefore, upregulation of integrin 7ß1 compensates as a transmembrane muscle cell attachment for sarcoglycan consistent with overlapping roles for sarcoglycan and integrins in mediating cytoskeletal-membrane-extracellular matrix interaction.
Key words: Sarcoglycan, Dystrophin, Integrin, Membrane, Muscle
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