[Effect of the mutation muscular dysgenesis on the mitochondrial metabolism of fibroblasts in vitro].

Abstract excerpt

Muscular dysgenesis is a lethal mutation characterized by an absence of striated muscle contraction. This abnormality of function caused by a defect in excitation-contraction coupling is due to the lack of the alpha 1 subunit from the dihydropyridine receptor in the transverse tubule membrane. A phenotypical reversion is obtained in vitro by addition of normal cells, as fibroblasts, in dysgenic muscle culture....

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