Neuronal nitric oxide synthase localizes to utrophin expressing intercalated discs and stabilizes their structural integrity

Highlights • nNOS, sGCβ1 and utrophin localize to intercalated discs. • nNOS is mislocalized in mdx and mdx:utr mice. • nNOSµ KO and mdx:nNOSµ KO mice show mislocalized residual nNOS. • Intercalated discs are disrupted in mdx, mdx:utr, nNOSµ KO and mdx:nNOSµ KO mice. • mdx:nNOSµ, KO but not mdx or n...

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Veröffentlicht in:	Neuromuscular disorders : NMD 2015-12, Vol.25 (12), p.964-976
Hauptverfasser:	Gonzalez, J. Patrick, Crassous, Pierre-Antoine, Schneider, Joel S, Beuve, Annie, Fraidenraich, Diego
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Cardiomyopathy Duchenne muscular dystrophy Guanylate Cyclase - metabolism Intercalated discs Mice Mice, Inbred C57BL Mice, Inbred mdx Mice, Knockout Myocytes, Cardiac - enzymology Myocytes, Cardiac - pathology Neurology Nitric oxide Nitric oxide synthase Nitric Oxide Synthase Type I - genetics Nitric Oxide Synthase Type I - metabolism Receptors, Cytoplasmic and Nuclear - metabolism Sarcolemma - enzymology Soluble Guanylyl Cyclase Utrophin Utrophin - genetics Utrophin - metabolism
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Zusammenfassung:	Highlights • nNOS, sGCβ1 and utrophin localize to intercalated discs. • nNOS is mislocalized in mdx and mdx:utr mice. • nNOSµ KO and mdx:nNOSµ KO mice show mislocalized residual nNOS. • Intercalated discs are disrupted in mdx, mdx:utr, nNOSµ KO and mdx:nNOSµ KO mice. • mdx:nNOSµ, KO but not mdx or nNOSµ KO mice develop premature cardiac fibrosis.
ISSN:	0960-8966 1873-2364
DOI:	10.1016/j.nmd.2015.09.011