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Metabolic crisis and TRPM4 activation cause QT prolongation in TANGO2 deficiency disorder
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DOI：10.1093/cvr/cvag143.png)
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Abstract
En 中文
TANGO2 deficiency disorder (TDD), caused by homozygous large deletions 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.
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