Sarcolemmal ion channels in dystrophin-deficient skeletal muscle fibres

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Abstract

Duchenne muscular dystrophy (DMD) is a genetic disease caused by mutations in the dystrophin gene and characterized by progressive skeletal muscle degeneration. A current hypothesis suggests that degeneration of dystrophin-deficient skeletal muscle results from a chronic intracellular Ca2+ overload. Ca2+ handling in skeletal muscle is tightly controlled by the membrane potential which is set by sarcolemmal ion channels activity. Also, with regard to the subsarcolemmal localization of dystrophin, it is reasonable to enquire if the distribution and function of ion channels might be affected by the absence of dystrophin. This paper briefly summarizes the current knowledge of the properties of sarcolemmal ion channels in fully differentiated dystrophin-deficient skeletal muscle fibres.

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