Neuronal nitric oxide synthase localizes to utrophin expressing intercalated discs and stabilizes their structural integrity
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- 作者：J. Patrick Gonzaleza ; Pierre-Antoine Crassousb ; Joel S. Schneidera ; 1 ; Annie Beuveb ; Diego Fraidenraicha ; fraidedi@rutgers.edu" class="auth_mail" title="E-mail the corresponding author
- 关键词：Duchenne muscular dystrophy ; Nitric oxide synthase ; Nitric oxide ; Utrophin ; Intercalated discs ; Cardiomyopathy
- 刊名：Neuromuscular Disorders
- 出版年：2015
- 出版时间：December 2015
- 年：2015
- 卷：25
- 期：12
- 页码：964-976
- 全文大小：7477 K
文摘
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nNOS, sGCβ1 and utrophin localize to intercalated discs.
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nNOS is mislocalized in mdx and mdx:utr mice.
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nNOSµ KO and mdx:nNOSµ KO mice show mislocalized residual nNOS.
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Intercalated discs are disrupted in mdx, mdx:utr, nNOSµ KO and mdx:nNOSµ KO mice.
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mdx:nNOSµ, KO but not mdx or nNOSµ KO mice develop premature cardiac fibrosis.