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Article

LQT1 mutations in KCNQ1 C-terminus assembly domain suppress IKs using different mechanisms.

Cardiovascular research - 1 Dec 2014

Aromolaran Ademuyiwa S, Subramanyam Prakash, Chang Donald D, Kobertz William R, Colecraft Henry M

Abstract excerpt

AIMS: Long QT syndrome 1 (LQT1) mutations in KCNQ1 that decrease cardiac IKs (slowly activating delayed rectifier K(+) current) underlie ventricular arrhythmias and sudden death. LQT1 mutations may suppress IKs by preventing KCNQ1 assembly, disrupting surface trafficking, or inhibiting gating. We investigated mechanisms underlying how three LQT1 mutations in KCNQ1 C-terminus assembly domain (R555H/G589D/L619M)...

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Source

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Identifiers

PMID: 25344363
DOI: 10.1093/cvr/cvu231
PMCID: PMC4296111

Genes

KCNQ1

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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