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Article

Allele-specific RNA interference rescues the long-QT syndrome phenotype in human-induced pluripotency stem cell cardiomyocytes.

European heart journal - 1 Apr 2014

Matsa Elena, Dixon James E, Medway Christopher, Georgiou Orestis, Patel Minal J, Morgan Kevin, Kemp Paul J, Staniforth Andrew, Mellor Ian, Denning Chris

Abstract excerpt

AIMS: Long-QT syndromes (LQTS) are mostly autosomal-dominant congenital disorders associated with a 1:1000 mutation frequency, cardiac arrest, and sudden death. We sought to use cardiomyocytes derived from human-induced pluripotency stem cells (hiPSCs) as an in vitro model to develop and evaluate gene-based therapeutics for the treatment of LQTS. METHODS AND RESULTS: We produced LQTS-type 2 (LQT2) hiPSC...

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Source

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Identifiers

PMID: 23470493
DOI: 10.1093/eurheartj/eht067
PMCID: PMC3992427

Genes

KCNH2

Variants

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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