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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Bloch, R. J. Williams, M. W. |
| Description | Author Affiliation: Williams MW ( Department of Physiology, School of Medicine, University of Maryland, Baltimore, Maryland 21201, USA.) |
| Abstract | We used immunofluorescence techniques and confocal imaging to study the organization of the membrane skeleton of skeletal muscle fibers of mdx mice, which lack dystrophin. β-Spectrin is normally found at the sarcolemma in costameres, a rectilinear array of longitudinal strands and elements overlying Z and M lines. However, in the skeletal muscle of mdx mice, β-spectrin tends to be absent from the sarcolemma over M lines and the longitudinal strands may be disrupted or missing. Other proteins of the membrane and associated cytoskeleton, including syntrophin, β-dystroglycan, vinculin, and Na,K-ATPase are also concentrated in costameres, in control myofibers, and mdx muscle. They also distribute into the same altered sarcolemmal arrays that contain β-spectrin. Utrophin, which is expressed in mdx muscle, also codistributes with β-spectrin at the mutant sarcolemma. By contrast, the distribution of structural and intracellular membrane proteins, including α-actinin, the Ca-ATPase and dihydropyridine receptors, is not affected, even at sites close to the sarcolemma. Our results suggest that in myofibers of the mdx mouse, the membrane- associated cytoskeleton, but not the nearby myoplasm, undergoes widespread coordinated changes in organization. These changes may contribute to the fragility of the sarcolemma of dystrophic muscle. |
| ISSN | 00219525 |
| e-ISSN | 15408140 |
| Journal | The Journal of Cell Biology |
| Issue Number | 6 |
| Volume Number | 144 |
| Language | English |
| Publisher | Rockefeller University Press (United States) |
| Publisher Date | 1999-03-22 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | Muscle, Skeletal Metabolism Pathology Muscular Dystrophy, Animal Animals Calcium-Transporting ATPases Cytoskeletal Proteins Dystrophin Deficiency Genetics Fluorescent Antibody Technique Membrane Proteins Mice Mice, Inbred Mdx Microscopy, Confocal Sarcolemma Sodium-Potassium-Exchanging ATPase Spectrin Utrophin 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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