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Mechanisms of pharmacological rescue of trafficking defective hERG mutant channels in human long QT syndrome

Long QT syndrome type 2 is caused by mutations in the human ether-a-go-go-related gene (hERG). We previously reported that the N470D mutation is retained in the endoplasmic reticulum (ER) but can be rescued to the plasma membrane by hERG channel blocker E-4031. The mechanisms of ER retention and how...

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Autori principali: Gong, Qiuming, Jones, Melanie A., Zhou, Zhengfeng
Natura: Artigo
Lingua:Inglês
Pubblicazione: 2005
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC1624912/
https://ncbi.nlm.nih.gov/pubmed/16361248
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M511765200
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