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Long QT syndrome-associated mutations in the S4-S5 linker of KvLQT1 potassium channels modify gating and interaction with minK subunits.

The Journal of biological chemistry - 23 Jul 1999

Franqueza L, Lin M, Shen J, Splawski I, Keating M T, Sanguinetti M C

Abstract excerpt

Long QT syndrome is an inherited disorder of cardiac repolarization caused by mutations in cardiac ion channel genes, including KVLQT1. In this study, the functional consequences of three long QT-associated missense mutations in KvLQT1 (R243C, W248R, E261K) were characterized using the Xenopus oo...

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Electrophysiology
Ion Channel Gating
KCNQ Potassium Channels
KCNQ1 Potassium Channel
Long QT Syndrome

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Potassium Channels

Potassium Channels, Voltage-Gated

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PMID: 10409658
DOI: 10.1074/jbc.274.30.21063

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 31 Aug 2026. Prepared record publication-10409658-2026-08-31.1. 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 S4-S5 linker of KvLQT1 potassium channels modify gating and interaction with minK subunits.PMID 10409658Read ↗

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

KvLQT1 and IsK (minK) proteins associate to form the IKS cardiac potassium currentSemantic proximity Read ↗

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