Repair by Satellite

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Science  20 Sep 2002:
Vol. 297, Issue 5589, pp. 1959
DOI: 10.1126/science.297.5589.1959a

Several forms of muscular dystrophy are caused by mutations that disrupt the dystrophin-glycoprotein complex, a critical assembly of muscle proteins that links the cytoskeleton to the extracellular matrix. Cohn et al. investigated the function of dystroglycan, a major component of this complex, by generating mice in which expression of the protein was selectively ablated in differentiated skeletal muscle. Surprisingly, although these mice showed signs of skeletal muscle cell death within 6 weeks after birth, they developed only a mild form of muscular dystrophy. Disease progression was prevented by the reparative action of satellite cells, a resident population of undifferentiated cells in muscle that retained expression of dystroglycan in the mutant mice. This finding indicates that satellite cells play an important role in the pathogenesis of muscular dystrophy and offers hope that the progression of this and other muscle wasting disorders can be slowed by therapeutic strategies designed to maintain muscle regenerative capacity. — PAK

Cell110, 639 (2002).

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