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Article

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

The Journal of biological chemistry - 17 Feb 2006

Gong Qiuming, Jones Melanie A, Zhou Zhengfeng

Abstract excerpt

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 E-4031 rescues the N470D mutant are poorly understood. In this study, we investigated the interaction of hERG...

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Identifiers

PMID: 16361248
DOI: 10.1074/jbc.M511765200
PMCID: PMC1624912

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

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Mechanisms of pharmacological rescue of trafficking-defective hERG mutant channels in human long QT syndrome.PMID 16361248Read ↗

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