| First Author | Dunckley MG | Year | 1994 |
| Journal | J Cell Sci | Volume | 107 ( Pt 6) |
| Pages | 1469-75 | PubMed ID | 7962190 |
| Mgi Jnum | J:19097 | Mgi Id | MGI:67297 |
| Doi | 10.1242/jcs.107.6.1469 | Citation | Dunckley MG, et al. (1994) Independent localization of dystrophin N- and C-terminal regions to the sarcolemma of mdx mouse myofibres in vivo. J Cell Sci 107(Pt 6):1469-75 |
| abstractText | Dystrophin has been proposed to associate with the skeletal muscle membrane by way of a glycoprotein complex that interacts with its C-terminal domains. Transfection of mdx mouse myotubes in culture or myofibres in vivo with recombinant genes encoding human dystrophin deletion mutants shows, however, that not only the C terminus of dystrophin but also its N-terminal actin-binding domain can locate independently to the muscle sarcolemma. This observation suggests that lack of sarcolemma-associated dystrophin in Duchenne muscular dystrophy (DMD) muscle may result from enhanced degradation of truncated mutation products rather than their inability per se to associate with the sarcolemma. |
