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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 of ventricular action potentials. In this study, we used the oocyte expression system and voltage clamp techniques to...

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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 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 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 ↗

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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 ↗

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

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