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Article

Mechanisms of disease pathogenesis in long QT syndrome type 5.

American journal of physiology. Cell physiology - 1 Feb 2010

Harmer Stephen C, Wilson Andrew J, Aldridge Robert, Tinker Andrew

Abstract excerpt

KCNE1 associates with the pore-forming alpha-subunit KCNQ1 to generate the slow (I(Ks)) current in cardiac myocytes. Mutations in either KCNQ1 or KCNE1 can alter the biophysical properties of I(Ks) and mutations in KCNE1 underlie cases of long QT syndrome type 5 (LQT5). We previously investigated a mutation in KCNE1, T58P/L59P, which causes severe attenuation of I(Ks). However, how T58P/L59P acts to disrupt I(Ks)...

Topics

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Cricetulus
Endoplasmic Reticulum
KCNQ1 Potassium Channel
Long QT Syndrome
Membrane Potentials

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Multiprotein Complexes

Potassium Channels, Voltage-Gated

Source

Open in PubMed Open full text

Identifiers

PMID: 19907016
DOI: 10.1152/ajpcell.00308.2009
PMCID: PMC2822495

Genes

KCNQ1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026.

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Mechanisms of disease pathogenesis in long QT syndrome type 5.PMID 19907016Read ↗

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