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JCS ePress
online publication date 13 Jul 2004
doi: 10.1242/jcs.01234
Research Article
Genetic compensation for sarcoglycan loss by integrin 
7
1 in muscle
Michael J. Allikian,
Andrew A. Hack,
Stephanie Mewborn,
Ulrike Mayer,
and
Elizabeth M. McNally*
* Author for correspondence (e-mail: emcnally{at}medicine.bsd.uchicago.edu)
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 
7
1 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 71 compensates as a transmembrane muscle cell attachment for sarcoglycan consistent with overlapping roles for sarcoglycan and integrins in mediating cytoskeletal-membrane-extracellular matrix interaction.
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