Restoration of normal ultrastructure after expression of the alpha 1 subunit of the L-type Ca2+ channel in dysgenic myotubes

FEBS Letters
S Seigneurin-VeninL Garcia

Abstract

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.

References

Aug 1, 1989·Pflügers Archiv : European journal of physiology·R BournaudF Rieger
Apr 1, 1989·Proceedings of the National Academy of Sciences of the United States of America·G RomeyM Lazdunski
Sep 1, 1993·Mammalian Genome : Official Journal of the International Mammalian Genome Society·B DrouetM Pinçon-Raymond
Feb 1, 1993·Developmental Biology·N Chaudhari, K G Beam
Dec 6, 1963·Science·S GLUECKSOHN-WAELSCH

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