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Article

Isoform-specific dominant-negative effects associated with hERG1 G628S mutation in long QT syndrome.

PloS one - 1 Jan 2012

Stump Matthew R, Gong Qiuming, Zhou Zhengfeng

Abstract excerpt

BACKGROUND: Mutations in the human ether-a-go-go-related gene 1 (hERG1) cause type 2 long QT syndrome (LQT2). The hERG1 gene encodes a K(+) channel with properties similar to the rapidly activating delayed rectifying K(+) current in the heart. Several hERG1 isoforms with unique structural and functional properties have been identified. To date, the pathogenic mechanisms of LQT2 mutations have been predominantly...

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Source

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Identifiers

PMID: 22876326
DOI: 10.1371/journal.pone.0042552
PMCID: PMC3411645

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Source receipt: pubmed, retrieved 7 Sept 2026.

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