Stem Cell Based Therapy for Muscular Dystrophies: Cell Types and Environmental Factors Influencing Their Efficacy
نویسندگان
چکیده
Muscular dystrophies are inherited disorders in which muscle fibers are unusually susceptible to damage, leading to progressive loss of muscle structure and function. Some types of muscular dystrophy affect heart muscles, other involuntary muscles and other organs. The most common form of muscular dystrophy, Duchenne Muscular Dystrophy (DMD), is due to genetic deficiency of the protein dystrophin (Monaco et al., 1985). This protein is one of several partners that interact to link intracellular cytoskeleton to extracellular matrix (ECM) hence consolidating the scaffold necessary for maintaining structural integrity of skeletal muscle fibers. Dystrophin deficiency destabilizes muscle fibers, which become less resistant to contractions leading to muscle fiber necrosis and subsequent regeneration.
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