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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Rafael, J. A. Campbell, K. P. Straub, V. Chamberlain, J. S. |
| Description | Author Affiliation: Straub V ( Department of, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA.) |
| Abstract | 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 α2-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. |
| ISSN | 00219525 |
| e-ISSN | 15408140 |
| Journal | The Journal of Cell Biology |
| Issue Number | 2 |
| Volume Number | 139 |
| Language | English |
| Publisher | Rockefeller University Press (United States) |
| Publisher Date | 1997-10-20 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | Muscle Fibers, Skeletal Pathology Muscle, Skeletal Muscular Dystrophies Muscular Dystrophy, Animal Sarcolemma Animals Dystrophin Biosynthesis Deficiency Genetics Evans Blue Mice Mice, Inbred Mdx Mice, Mutant Strains Mice, Transgenic Mutagenesis Mutation Myocardium Serum Albumin Comparative Study Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. Cell Biology |
| Content Type | Text |
| Resource Type | Article |
| Subject | Cell Biology Medicine |
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