|
The
dystrophin-glycoprotein complex (DGC) helps stabilize the plasma membrane of
heart and skeletal muscle. Mutations in the genes encoding dystrophin
or the sarcoglycan subunits lead to cardiomyopathy and muscular dystrophy in
humans and in mouse models. The
DGC has both mechanical and signaling functions. The DGC links to the extracellular matrix through
dystroglycan and links to the cytoskeleton through dystrophin, dystroglycan
and through sarcoglycan.
References:
Hack,
A. A., C. T. Ly, F. Jiang, C. Clendenin, K. Sigrist, R. Wollman, and E. M.
McNally. g-sarcoglycan deficiency leads
to muscle membrane defects and apoptosis independent of dystrophin (1998) J. Cell Biol.
142:1279-1287.
Hack,
A.A., L. Cordier, D. I. Shoturma, M. Y. Lam, H. L. Sweeney and E. M. McNally.
(1999) Muscle degeneration
without mechanical injury in sarcoglycan-deficiency. Proc. NatŐl Acad. Sci., USA 96:10723-10728.
Thompson,
T., Y. M. Chan, A.A. Hack, M. Brosius, M. Rajala, H.G.W. Lidov, E. M.
McNally, S. Watkins and L.M. Kunkel.
Filamin 2 (FLN2): a muscle specific sarcoglycan interacting protein.
(2000) J. Cell Biol.
148:115-126.
Zhu,
X., M. E. Groh, M. Hadhazy, M. T. Wheeler, R. Wollman and E. M. McNally. Overexpression of g-sarcoglycan produces muscular
dystrophy: implications for sarcoglycan assembly. (2001) J.
Biol. Chem. 276:21785-90.
Allikian,
M., A. A. Hack, S. Mewborn, U. Mayer and E. M. McNally. Integrin is a compensatory
transmembrane linkage to sarcoglycan.
(in press, J. Cell Science)
|
