Article
Pharmacological rescue of trafficking defective HERG channels formed by coassembly of wild-type and long QT mutant N470D subunits.
American journal of physiology. Heart and circulatory physiology - 1 Aug 2004
Gong Qiuming, Anderson Corey L, January Craig T, Zhou Zhengfeng
Abstract excerpt
Mutations in the human ether-a-go-go-related gene (HERG) cause long QT syndrome. We previously showed that the HERG N470D mutation expressed as homotetrameric channels causes a protein trafficking defect, and this can be corrected by the HERG channel blocking drug E-4031. The N470D mutant also has been reported to cause dominant negative suppression of HERG current when coexpressed with wild-type channel...
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