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Article

An LQTS6 MiRP1 mutation suppresses pacemaker current and is associated with sinus bradycardia.

Journal of cardiovascular electrophysiology - 1 Sept 2013

Nawathe Pooja A, Kryukova Yelena, Oren Ronit V, Milanesi Raffaella, Clancy Colleen E, Lu Jonathan T, Moss Arthur J, Difrancesco Dario, Robinson Richard B

Abstract excerpt

BACKGROUND: Sinus node (SN) dysfunction is observed in some long-QT syndrome (LQTS) patients, but has not been studied as a function of LQTS genotype. LQTS6 involves mutations in the hERG β-subunit MiRP1, which also interacts with hyperpolarization-activated, cyclic nucleotide gated (HCN) channels-the molecular correlate of SN pacemaker current (If ). An LQTS registry search identified a 55-year male with M54T...

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Source

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Identifiers

PMID: 23631727
DOI: 10.1111/jce.12163
PMCID: PMC4059362

Genes

HCN4
HCN2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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