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Article

Silencing the Mutant KCNH2 Allele to Reduce the Effects of Long QT Syndrome Type 2.

Frontiers in bioscience (Landmark edition) - 12 Feb 2026

Wilders Ronald

Abstract excerpt

BACKGROUND: Long-QT syndrome type 2 (LQTS2), which is associated with life-threatening cardiac arrhythmias, is caused by pathogenic heterozygous loss-of-function mutations in the KCNH2 gene. This gene encodes the pore-forming Kv11.1 α-subunit of the ion channel that carries the rapid delayed rectifier potassium current (IKr). Pathogenic loss-of-function mutations reduce the amplitude of IKr, thereby prolonging...

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Source

Identifiers

PMID: 41761963
DOI: 10.31083/FBL49097

Genes

KCNH2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026.

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