[Effect of the mutation muscular dysgenesis on the mitochondrial metabolism of fibroblasts in vitro].
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. Perisynaptic fibroblasts from denervated muscle have been shown to synthetize molecules which are usually concentrated at the synapses suggesting that signal arising after denervation may influence fibroblasts synthesis ability. We show here that the energetic metabolism level from dysgenic fibroblast is lower than that from normal fibroblasts. The synthesis or the release of muscular signals that are involved in fibroblast differentiation could be disturbed in mdg/mdg myotubes.
Aymard
C
C
Laboratoire de Neurobiologie Cellulaire, CNRS URA 1126, Université de Bordeaux II, Talence, France.
Malgat
M
M
Mazat
J P
JP
Koenig
J
J
D004579:Electron Transport; D005347:Fibroblasts / Q000378:metabolism; D066298:In Vitro Techniques; D008931:Mitochondria, Muscle / Q000378:metabolism*; D009119:Muscle Contraction; D009132:Muscles / Q000002:abnormalities; D009135:Muscular Diseases / Q000235:genetics*; D009154:Mutation; D010101:Oxygen Consumption
Effet de la mutation muscular dysgenesis sur le métabolisme mitochondrial de fibroblastes in vitro.