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Article

Novel characteristics of a trafficking-defective G572R-hERG channel linked to hereditary long QT syndrome.

The Canadian journal of cardiology - 1 Oct 2010

Lian Jiangfang, Huang Na, Zhou Junbo, Ge Shijun, Huang Xiaoyan, Huo Jianhua, Liu Liying, Xu Weifeng, Zhang Shun, Yang Xi, Zhou Jianqing, Huang Chen

Abstract excerpt

BACKGROUND: The congenital long QT syndrome is a heterogeneous genetic disease associated with delayed cardiac repolarization, prolonged QT intervals, the development of ventricular arrhythmias and sudden death. Type 2 congenital long QT syndrome (LQT2) results from KCNH2 or hERG gene mutations. hERG encodes the K(v)11.1 alpha subunit of the rapidly activating delayed rectifier K(+) current in the heart. Studies...

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Source

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Identifiers

PMID: 20931094
DOI: 10.1016/s0828-282x(10)70439-6
PMCID: PMC2954534

Genes

KCNH2

Provenance

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

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

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Novel characteristics of a trafficking-defective G572R-hERG channel linked to hereditary long QT syndrome.PMID 20931094Read ↗

Partially dominant mutant channel defect corresponding with intermediate LQT2 phenotype.Semantic proximity Read ↗

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Novel mechanism associated with an inherited cardiac arrhythmia: defective protein trafficking by the mutant HERG (G601S) potassium channel.Semantic proximity Read ↗

Trafficking defect and proteasomal degradation contribute to the phenotype of a novel KCNH2 long QT syndrome mutation.Semantic proximity Read ↗

Functional characterization of the LQT2-causing mutation R582C and the associated voltage-dependent fluorescence signal.Semantic proximity Read ↗

Molecular pathogenesis of long QT syndrome type 2Semantic proximity Read ↗

Defective protein trafficking in hERG-associated hereditary long QT syndrome (LQT2): molecular mechanisms and restoration of intracellular protein processing.Semantic proximity Read ↗

Long QT Syndrome Type 2: Emerging Strategies for Correcting Class 2 KCNH2 (hERG) Mutations and Identifying New PatientsSemantic proximity Read ↗

Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-deficient) mechanism.Semantic proximity Read ↗

Combined gating and trafficking defect in Kv11.1 manifests as a malignant long QT syndrome phenotype in a large Danish p.F29L founder family.Semantic proximity Read ↗

Long QT syndrome with a high mortality rate caused by a novel G572R missense mutation in KCNH2.Semantic proximity Read ↗

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