title: Altered cross-bridge properties in skeletal muscle dystrophies.
abstract: 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.
authors.affiliation: Service de Cardiologie, Hôpital Henri Mondor, University Paris-Est Créteil Créteil, France ; Equipe 8, Institut National de la Santé et de la Recherche Médicale Créteil, France.
authors.forename: Aziz
authors.initials: A
authors.lastname: Guellich
authors.affiliation: UMRS 974, Institut National de la Santé et de la Recherche Médicale Paris, France ; UM 76, Université Pierre et Marie Curie, Sorbonne Universités Paris, France ; UMR 7215, Centre National de la Recherche Scientifique Paris, France ; Institut de Myologie Paris, France.
authors.forename: Elisa
authors.initials: E
authors.lastname: Negroni
authors.affiliation: Institut de Myologie Paris, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Decostre
authors.affiliation: UMRS 974, Institut National de la Santé et de la Recherche Médicale Paris, France ; UM 76, Université Pierre et Marie Curie, Sorbonne Universités Paris, France ; UMR 7215, Centre National de la Recherche Scientifique Paris, France ; Institut de Myologie Paris, France ; Assistance Publique-Hopitaux de Paris, Service de Pneumologie et Reanimation Medicale Paris, France.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Demoule
authors.affiliation: UMRS 974, Institut National de la Santé et de la Recherche Médicale Paris, France ; UM 76, Université Pierre et Marie Curie, Sorbonne Universités Paris, France ; UMR 7215, Centre National de la Recherche Scientifique Paris, France ; Institut de Myologie Paris, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Coirault
keywords: cross-bridge kinetics; muscle dystrophy; myopathies; myosin; skeletal muscle