Dystrophin-dependent efficiency of metabolic pathways in mouse skeletal muscles |
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Authors: | A E Chinet P C Even A Decrouy |
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Institution: | (1) Département de Physiologie, Centre Médical Universitaire, 1 rue Michel-Servet, 1211 Geneva 4, (Switzerland);(2) Laboratoire de Neurobiologie des Régulations, C.N.R.S. U.S.A. 637, Collège de France, 11 place Marcelin-Berthelot, 75231 Paris Cédex 05, (France) |
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Abstract: | Muscles from themdx mouse (X-linked genetic disorder similar to Duchenne muscular dystrophy) lack dystrophin-associated transsarcolemmal proteins1 and show reduced maintenance metabolic rates2. Here, microcalorimetric comparisons of metabolic stimulation by exogenous substrates in isolated muscles revealed substrate-selective limitation of chemical reaction rates through both glycolytic and TCA-cycle pathways, identical in slow- and fast-twitchmdx muscles. This systemic approach, as opposed to comparisons of single-enzyme activities, sheds new light on the function of dystrophin and associated proteins. The in vivo efficiency of metabolic pathways may depend on stabilization of enzyme complexes by dystrophin-associated elements of the cytoskeleton. |
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Keywords: | Muscular dystrophy skeletal muscle energy metabolism subcellular compartmentation substrate channelling cytoskeleton dystrophin |
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