title: Membrane Injury and Repair in the Muscular Dystrophies.
abstract: Muscle cells have an elaborate plasma membrane and t-tubule system that has been evolutionarily refined to maximize electrical conductivity for synchronous muscle contraction. However, this elaborate plasma membrane network has intrinsic vulnerabilities to stretch-induced membrane injury, and thus requires ongoing maintenance and repair. Herein we discuss the types of membrane injuries encountered by myofibers in healthy muscle and in muscular dystrophy. We review the different mechanisms by which muscle fibers in patients with muscular dystrophy are rendered more susceptible to injury, and we summarize the latest developments in our understanding of how the muscular dystrophy protein dysferlin mediates satellite-cell independent membrane repair.
authors.affiliation: Institute for Neuroscience and Muscle Research, Kids Research Institute, The Children's Hospital at Westmead, Sydney, New South Wales, Australia Discipline of Paediatrics and Child Health, Faculty of Medicine, University of Sydney, New South Wales, Australia sandra.cooper@sydney.edu.au.
authors.forename: Sandra T
authors.initials: ST
authors.lastname: Cooper
authors.affiliation: School of Medical Sciences, University of New South Wales, Sydney, New South Wales, Australia.
authors.forename: Stewart I
authors.initials: SI
authors.lastname: Head
mesh_terms: D000818:Animals; D002462:Cell Membrane / Q000378:metabolism*; D006801:Humans; D008565:Membrane Proteins / Q000378:metabolism; D018485:Muscle Fibers, Skeletal / Q000378:metabolism*; D009124:Muscle Proteins / Q000378:metabolism; D018482:Muscle, Skeletal / Q000293:injuries / Q000378:metabolism*; D009136:Muscular Dystrophies / Q000378:metabolism*
chemical_list: D008565:Membrane Proteins; D009124:Muscle Proteins
keywords: membrane repair; muscle damage; muscular dystrophy; skeletal muscle