Sarcolemmal ion channels in dystrophin-deficient skeletal muscle fibres
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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.
KeywordsDystrophin Duchenne muscular dystrophy mdx mouse Skeletal muscle fibre Ion channels Electrophysiology Patch clamp Voltage clamp Calcium
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This work was supported by grants from the Université Lyon 1, the Centre National de la Recherche Scientifique (CNRS), the Association Française contre les Myopathies and the Agence Nationale de la Recherche. I thank Dr. V. Jacquemond for helpful comments on the manuscript.
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