Absence of Dystrophin Disrupts Skeletal Muscle Signaling: Roles of Ca2+, Reactive Oxygen Species, and Nitric Oxide in the Development of Muscular Dystrophy

• Published in: Physiological reviews Vol. 96; no. 1; pp. 253 - 305
• Main Authors: Allen, David G, Whitehead, Nicholas P, Froehner, Stanley C
• Format: Journal Article
• Language: English
• Published: United States American Physiological Society 01.01.2016
• Subjects: Animals; Calcium - metabolism; Calcium Signaling; Dystrophin - deficiency; Dystrophin - genetics; Dystrophin - metabolism; Gene Expression Regulation; Humans; Muscle Development; Muscle, Skeletal - metabolism; Muscle, Skeletal - pathology; Muscle, Skeletal - physiopathology; Muscular Dystrophies - genetics; Muscular Dystrophies - metabolism; Muscular Dystrophies - pathology; Muscular Dystrophies - physiopathology; Nitric Oxide - metabolism; Reactive Oxygen Species - metabolism; Regeneration; Reviews; Signal Transduction

Dystrophin is a long rod-shaped protein that connects the subsarcolemmal cytoskeleton to a complex of proteins in the surface membrane (dystrophin protein complex, DPC), with further connections via laminin to other extracellular matrix proteins. Initially considered a structural complex that protec...