| First Author | Seigneurin-Venin S | Year | 1994 |
| Journal | FEBS Lett | Volume | 342 |
| Issue | 2 | Pages | 129-34 |
| PubMed ID | 8143864 | Mgi Jnum | J:18533 |
| Mgi Id | MGI:66718 | Doi | 10.1016/0014-5793(94)80486-9 |
| Citation | Seigneurin-Venin S, et al. (1994) Restoration of normal ultrastructure after expression of the alpha 1 subunit of the L-type Ca2+ channel in dysgenic myotubes. FEBS Lett 342(2):129-34 |
| abstractText | Muscular dysgenesis (mdg) is a spontaneous mutation affecting the alpha 1 subunit of the skeletal L-type Ca2+ channel. mdg/mdg mice suffer from a skeletal muscle disease characterised by low levels of the slow Ca2+ current, lack of contractile activity, and immature organisation of skeletal muscle. Microinjections of a cDNA encoding alpha 1 into mutant myotubes restore excitation-contraction coupling. We checked here that dysgenic myotubes transfected with expression vectors, including a full-length alpha 1 cDNA, also recover normal ultrastructural features. Transfection of alpha 1 cDNA partially deleted on the 5' end leads to the recovery of a good structural organisation without any improvement in the mutant physiological phenotype. These results suggest that: (i) the proper expression of alpha 1 is required for the full muscle differentiation of muscular dysgenesis myotubes, and (ii) portions of the alpha 1 molecule may be involved in the structural organisation of a muscle fiber, independent of its known functional properties. |
