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Altered cross-bridge properties in skeletal muscle dystrophies

Abstract : † Co-first authors. Force and motion generated by skeletal muscle ultimately depends on the cyclical interaction of actin with myosin. This mechanical process is regulated by intracellular Ca 2+ through the thin filament-associated regulatory proteins i.e.; troponins and tropomyosin. Muscular dystrophies are a group of heterogeneous genetic affections characterized by progressive degeneration and weakness of the skeletal muscle as a consequence of loss of muscle tissue which directly reduces the number of potential myosin cross-bridges involved in force production. Mutations in genes responsible for skeletal muscle dystrophies (MDs) have been shown to modify the function of contractile proteins and cross-bridge interactions. Altered gene expression or RNA splicing or post-translational modifications of contractile proteins such as those related to oxidative stress, may affect cross-bridge function by modifying key proteins of the excitation-contraction coupling. Micro-architectural change in myofilament is another mechanism of altered cross-bridge performance. In this review, we provide an overview about changes in cross-bridge performance in skeletal MDs and discuss their ultimate impacts on striated muscle function.
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Aziz Guellich, Elisa Negroni, Valérie Decostre, Alexandre Demoule, Catherine Coirault. Altered cross-bridge properties in skeletal muscle dystrophies. Frontiers in Physiology, Frontiers, 2014, 5, ⟨10.3389/fphys.2014.00393⟩. ⟨inserm-02426461⟩



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