title: Nitric oxide synthase deficiency and the pathophysiology of muscular dystrophy.
abstract: The secondary loss of neuronal nitric oxide synthase (nNOS) that occurs in dystrophic muscle is the basis of numerous, complex and interacting features of the dystrophic pathology that affect not only muscle itself, but also influence the interaction of muscle with other tissues. Many mechanisms through which nNOS deficiency contributes to misregulation of muscle development, blood flow, fatigue, inflammation and fibrosis in dystrophic muscle have been identified, suggesting that normalization in NO production could greatly attenuate diverse aspects of the pathology of muscular dystrophy through multiple regulatory pathways. However, the relative importance of the loss of nNOS from the sarcolemma versus the importance of loss of total nNOS from dystrophic muscle remains unknown. Although most current evidence indicates that nNOS localization at the sarcolemma is not required to achieve NO-mediated reductions of pathology in muscular dystrophy, the question remains open concerning whether membrane localization would provide a more efficient rescue from features of the dystrophic phenotype.
authors.affiliation: Molecular, Cellular & Integrative Physiology Program, University of California, Los Angeles, CA, USA Department of Integrative Biology and Physiology, University of California, Los Angeles, CA, USA Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, University of California, Los Angeles, CA, USA jtidball@physci.ucla.edu.
authors.forename: James G
authors.initials: JG
authors.lastname: Tidball
authors.affiliation: Department of Integrative Biology and Physiology, University of California, Los Angeles, CA, USA.
authors.forename: Michelle
authors.initials: M
authors.lastname: Wehling-Henricks
mesh_terms: D000818:Animals; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics / Q000378:metabolism; D006801:Humans; D020388:Muscular Dystrophy, Duchenne / Q000201:enzymology* / Q000503:physiopathology*; D009569:Nitric Oxide / Q000378:metabolism; D052248:Nitric Oxide Synthase Type I / Q000172:deficiency*; D012508:Sarcolemma / Q000201:enzymology
chemical_list: D016189:Dystrophin; D009569:Nitric Oxide; D052248:Nitric Oxide Synthase Type I