Dystrophin-dependent efficiency of metabolic pathways in mouse skeletal muscles


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.

DOI: 10.1007/BF01921731

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@article{Chinet1994DystrophindependentEO, title={Dystrophin-dependent efficiency of metabolic pathways in mouse skeletal muscles}, author={A. E. Chinet and P. C. Even and A. Decrouy}, journal={Experientia}, year={1994}, volume={50}, pages={602-605} }