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Article

A long QT mutation substitutes cholesterol for phosphatidylinositol-4,5-bisphosphate in KCNQ1 channel regulation.

PloS one - 1 Jan 2014

Coyan Fabien C, Abderemane-Ali Fayal, Amarouch Mohamed Yassine, Piron Julien, Mordel Jérôme, Nicolas Céline S, Steenman Marja, Mérot Jean, Marionneau Céline, Thomas Annick, Brasseur Robert, Baró Isabelle, Loussouarn Gildas

Abstract excerpt

INTRODUCTION: Phosphatidylinositol-4,5-bisphosphate (PIP2) is a cofactor necessary for the activity of KCNQ1 channels. Some Long QT mutations of KCNQ1, including R243H, R539W and R555C have been shown to decrease KCNQ1 interaction with PIP2. A previous study suggested that R539W is paradoxically less sensitive to intracellular magnesium inhibition than the WT channel, despite a decreased interaction with PIP2. In...

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Identifiers

PMID: 24681627
DOI: 10.1371/journal.pone.0093255
PMCID: PMC3969324

Genes

KCNQ1

Variants

Provenance

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

A long QT mutation substitutes cholesterol for phosphatidylinositol-4,5-bisphosphate in KCNQ1 channel regulation.PMID 24681627Read ↗

Impaired KCNQ1-KCNE1 and phosphatidylinositol-4,5-bisphosphate interaction underlies the long QT syndrome.Semantic proximity Read ↗

Ligand sensitivity of type-1 inositol 1,4,5-trisphosphate receptor is enhanced by the D2594K mutation.Semantic proximity Read ↗

KCNE1 enhances phosphatidylinositol 4,5-bisphosphate (PIP2) sensitivity of IKs to modulate channel activity.Semantic proximity Read ↗

A single amino acid mutation attenuates rundown of voltage-gated calcium channels.Semantic proximity Read ↗

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Mutant MiRP1 subunits modulate HERG K+ channel gating: a mechanism for pro-arrhythmia in long QT syndrome type 6.Semantic proximity Read ↗

Structural determinants for GqPCR-mediated inhibition of TASK K2P K <sup>+</sup> channels and their dysfunction in diseaseSemantic proximity Read ↗

The second PI(3,5)P2 binding site in the S0 helix of KCNQ1 stabilizes PIP2-at the primary PI1 site with potential consequences on intermediate-to-open state transition.Semantic proximity Read ↗

Dual regulation of voltage-gated calcium channels by PtdIns(4,5)P2.Semantic proximity Read ↗

Disease-Linked Super-Trafficking of a Mutant Potassium ChannelSemantic proximity Read ↗

Phosphatidylinositol-4,5-bisphosphate is required for KCNQ1/KCNE1 channel function but not anterograde trafficking.Semantic proximity Read ↗

PIP(2) activates KCNQ channels, and its hydrolysis underlies receptor-mediated inhibition of M currents.Semantic proximity Read ↗

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