Gene KCNH2
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Also known as
HERG, LQT2, potassium channel Kv11.1, ether-a-go-go related geneOverview
KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2), also known as hERG, encodes a potassium channel critical for cardiac repolarization. The hERG channel conducts the rapid delayed rectifier potassium current (IKr) that terminates the cardiac action potential and determines the duration of the QT interval on ECG.Mutations in KCNH2 cause Long QT syndrome type 2 (LQT2), characterized by prolonged cardiac repolarization and risk of life-threatening arrhythmias including torsades de pointes and sudden cardiac death. The hERG channel is also the primary target for drug-induced QT prolongation, as many medications inadvertently block this channel, leading to acquired Long QT syndrome.
Understanding your KCNH2 genetic status is critical for cardiac health assessment and medication safety. Individuals with pathogenic variants require careful monitoring, avoidance of QT-prolonging drugs, and potentially prophylactic treatment with beta-blockers or implantable defibrillators.