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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Chinet, A.E., Even, P.C. & Decrouy, A. Dystrophin-dependent efficiency of metabolic pathways in mouse skeletal muscles. Experientia 50, 602–605 (1994). https://doi.org/10.1007/BF01921731
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DOI: https://doi.org/10.1007/BF01921731