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Article

Long QT syndrome-associated mutations in the Per-Arnt-Sim (PAS) domain of HERG potassium channels accelerate channel deactivation.

The Journal of biological chemistry - 9 Apr 1999

Chen J, Zou A, Splawski I, Keating M T, Sanguinetti M C

Abstract excerpt

Mutations in the human ether-a-go-go-related gene (HERG) cause long QT syndrome, an inherited disorder of cardiac repolarization that predisposes affected individuals to life-threatening arrhythmias. HERG encodes the cardiac rapid delayed rectifier potassium channel that mediates repolarization o...

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ERG1 Potassium Channel
Ether-A-Go-Go Potassium Channels
Helix-Loop-Helix Motifs
Long QT Syndrome
Potassium Channels

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Potassium Channels, Voltage-Gated

Transcriptional Regulator ERG

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Identifiers

PMID: 10187793
DOI: 10.1074/jbc.274.15.10113

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 31 Aug 2026. Prepared record publication-10187793-2026-08-31.1. Manifest publication-graph-cohort-2026-08-31.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 31 Aug 2026.

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Long QT syndrome-associated mutations in the Per-Arnt-Sim (PAS) domain of HERG potassium channels accelerate channel deactivation.PMID 10187793Read ↗

Molecular basis of slow activation of the human ether-a-go-go related gene potassium channel.Semantic proximity Read ↗

Dysfunction of delayed rectifier potassium channels in an inherited cardiac arrhythmia.Semantic proximity Read ↗

hERG K(+) channels: structure, function, and clinical significance.Semantic proximity Read ↗

The hERG K(+) channel S4 domain L532P mutation: characterization at 37°C.Semantic proximity Read ↗

Rescue of aberrant gating by a genetically encoded PAS (Per-Arnt-Sim) domain in several long QT syndrome mutant human ether-á-go-go-related gene potassium channels.Semantic proximity Read ↗

Structural basis of action for a human ether-a-go-go-related gene 1 potassium channel activator.Semantic proximity Read ↗

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

An Interdomain KCNH2 Mutation Produces an Intermediate Long QT Syndrome.Semantic proximity Read ↗

Molecular Basis of Cardiac Delayed Rectifier Potassium Channel Function and Pharmacology.Semantic proximity Read ↗

HERG, a human inward rectifier in the voltage-gated potassium channel family.Semantic proximity Read ↗

Human ether-a-go-go related gene (hERG) K+ channels: function and dysfunction.Semantic proximity Read ↗

Comparative effects of the short QT N588K mutation at 37 degrees C on hERG K+ channel current during ventricular, Purkinje fibre and atrial action potentials: an action potential clamp study.Semantic proximity Read ↗

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