Metabolic crisis and TRPM4 activation cause QT prolongation in TANGO2 deficiency disorder.

doi:10.1093/cvr/cvag143

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Article

Metabolic crisis and TRPM4 activation cause QT prolongation in TANGO2 deficiency disorder.

Cardiovascular research - 26 Aug 2026

Wang Lili, Kim Kyungsoo, Kannankeril Prince J, Zheng Chen, Blackwell Daniel J, Mackenzie Samuel J, Brookes Paul S, Knollmann Björn C

Abstract excerpt

AIMS: TANGO2 deficiency disorder (TDD), caused by biallelic pathogenic variants in TANGO2, is associated with impaired fatty acid oxidation and metabolic crises that frequently trigger QT prolongation and arrhythmias. The objective was to study the mechanisms responsible for QT prolongation and arrhythmogenesis in TDD. METHODS AND RESULTS: CRISPR/Cas9 were used to generate human-induced pluripotent stem cell...

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Identifiers

PMID: 42389941
DOI: 10.1093/cvr/cvag143
PMCID: PMC13508696

Genes

TANGO2
TRPM4

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026.