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.