Dystrophin-dependent efficiency of metabolic pathways in mouse skeletal muscles

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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.