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Article

The rate-dependent biophysical properties of the LQT1 H258R mutant are counteracted by a dominant negative effect on channel trafficking.

Journal of molecular and cellular cardiology - 1 Jun 2010

Labro Alain J, Boulet Inge R, Timmermans Jean-Pierre, Ottschytsch Natacha, Snyders Dirk J

Abstract excerpt

The long QT syndrome (LQTS) is a cardiac disorder caused by a prolonged ventricular repolarization. The co-assembly of the pore-forming human KCNQ1 alpha-subunits with the modulating hKCNE1 beta-subunits generates I(Ks)in vivo, explaining why mutations in the hKCNQ1 gene underlie the LQT1 form of...

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Source

Identifiers

PMID: 19913547
DOI: 10.1016/j.yjmcc.2009.10.027

Genes

KCNQ1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-19913547-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

Related research

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The rate-dependent biophysical properties of the LQT1 H258R mutant are counteracted by a dominant negative effect on channel trafficking.PMID 19913547Read ↗

Mechanisms of disease pathogenesis in long QT syndrome type 5.Semantic proximity Read ↗

Trafficking-deficient long QT syndrome mutation KCNQ1-T587M confers severe clinical phenotype by impairment of KCNH2 membrane localization: evidence for clinically significant IKr-IKs alpha-subunit interaction.Semantic proximity Read ↗

A dual mechanism for I(Ks) current reduction by the pathogenic mutation KCNQ1-S277L.Semantic proximity Read ↗

Microscopic mechanisms for long QT syndrome type 1 revealed by single-channel analysis of I(Ks) with S3 domain mutations in KCNQ1.Semantic proximity Read ↗

Elevated heart rate triggers action potential alternans and sudden death. translational study of a homozygous KCNH2 mutation.Semantic proximity Read ↗

Functional effects of a KCNQ1 mutation associated with the long QT syndrome.Semantic proximity Read ↗

Mutations in the hminK gene cause long QT syndrome and suppress IKs function.Semantic proximity Read ↗

Functionally Aberrant Mutant KCNQ1 With Intermediate Heterozygous and Homozygous Phenotypes.Semantic proximity Read ↗

A590T mutation in KCNQ1 C-terminal helix D decreases IKs channel trafficking and function but not Yotiao interaction.Semantic proximity Read ↗

Mechanisms of I(Ks) suppression in LQT1 mutants.Semantic proximity Read ↗

Functional Interactions between KCNE1 C-Terminus and the KCNQ1 ChannelSemantic proximity Read ↗

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

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