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Article

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

Circulation - 4 May 1999

Furutani M, Trudeau M C, Hagiwara N, Seki A, Gong Q, Zhou Z, Imamura S, Nagashima H, Kasanuki H, Takao A, Momma K, January C T, Robertson G A, Matsuoka R

Abstract excerpt

BACKGROUND: The congenital long-QT syndrome (LQTS) is an inherited disorder characterized by a prolonged cardiac action potential and a QT interval that leads to arrhythmia. Mutations in the human ether-a-go-go-related gene (HERG), which encodes the rapidly activating component of the delayed rectifier current (IKr), cause chromosome 7-linked LQTS (LQT2). Studies of mutant HERG channels in heterologous systems...

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Identifiers

PMID: 10226095
DOI: 10.1161/01.cir.99.17.2290

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Novel mechanism associated with an inherited cardiac arrhythmia: defective protein trafficking by the mutant HERG (G601S) potassium channel.PMID 10226095Read ↗

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

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

Long QT syndrome: cellular basis and arrhythmia mechanism in LQT2.Semantic proximity Read ↗

A new C-terminal hERG mutation A915fs+47X associated with symptomatic LQT2 and auditory-trigger syncope.Semantic proximity Read ↗

Biophysical characterization of the short QT mutation hERG-N588K reveals a mixed gain-and loss-of-function.Semantic proximity Read ↗

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

Long-QT syndrome-associated missense mutations in the pore helix of the HERG potassium channel.Semantic proximity Read ↗

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

Spectrum of HERG K+-channel dysfunction in an inherited cardiac arrhythmia.Semantic proximity Read ↗

The variant hERG/R148W associated with LQTS is a mutation that reduces current density on co-expression with the WT.Semantic proximity Read ↗

The mutation L69P in the PAS domain of the hERG potassium channel results in LQTS by trafficking deficiency.Semantic proximity Read ↗

A missense mutation (G604S) in the S5/pore region of HERG causes long QT syndrome in a Chinese family with a high incidence of sudden unexpected death.Semantic proximity Read ↗

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