J. Cell Biol.
© The Rockefeller University Press
0021-9525/97/10/375/11 $2.00
Volume 139, Number 2, October 20, 1997 375-385

Animal Models for Muscular Dystrophy Show Different Patterns of Sarcolemmal Disruption

Volker Straub,^* Jill A. Rafael,^§ Jeffrey S. Chamberlain,^§ and Kevin 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; and ^§ Department of Human Genetics, University of Michigan Medical School, Ann Arbor, Michigan 48109

Genetic defects in a number of components of the dystrophin-glycoprotein complex (DGC) lead to distinct forms of muscular dystrophy. However, little is known about how alterations in the DGC are manifested in the pathophysiology present in dystrophic muscle tissue. One hypothesis is that the DGC protects the sarcolemma from contraction-induced damage. Using tracer molecules, we compared sarcolemmal integrity in animal models for muscular dystrophy and in muscular dystrophy patient samples. Evans blue, a low molecular weight diazo dye, does not cross into skeletal muscle fibers in normal mice. In contrast, mdx mice, a dystrophin-deficient animal model for Duchenne muscular dystrophy, showed significant Evans blue accumulation in skeletal muscle fibers. We also studied Evans blue dispersion in transgenic mice bearing different dystrophin mutations, and we demonstrated that cytoskeletal and sarcolemmal attachment of dystrophin might be a necessary requirement to prevent serious fiber damage. The extent of dye incorporation in transgenic mice correlated with the phenotypic severity of similar dystrophin mutations in humans. We furthermore assessed Evans blue incorporation in skeletal muscle of the dystrophia muscularis (dy/dy) mouse and its milder allelic variant, the dy^2J/dy^2J mouse, animal models for congenital muscular dystrophy. Surprisingly, these mice, which have defects in the laminin 2-chain, an extracellular ligand of the DGC, showed little Evans blue accumulation in their skeletal muscles. Taken together, these results suggest that the pathogenic mechanisms in congenital muscular dystrophy are different from those in Duchenne muscular dystrophy, although the primary defects originate in two components associated with the same protein complex.

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• Barton, E. R., Morris, L., Musaro, A., Rosenthal, N., Sweeney, H. L. (2002). Muscle-specific expression of insulin-like growth factor I counters muscle decline in mdx mice. JCB 157: 137-148 [Abstract] [Full Text]  
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