Human ether-a-go-go related gene (HERG) encodes the pore-forming alpha subunit of the delayed rectifier potassium channel IKr. There are two N-terminal splice variants of HERG include the full-length isoform 1 alpha and the shorter isoform 1 beta. Isoform 1 beta lacks the PAS motif and deactivates at a faster rate than isoform 1alpha. Residues within the C-terminal play a role in channel expression and channel gating, including voltage-dependent activation. HERG is expressed in the heart and is more abundantly expressed in the ventricles than in the atria. Mutations in the gene encoding HERG increase beat-to-beat variability and early after depolarization. These fluctuations facilitate the genesis and propagation of premature heartbeats associated with inheritable long QT syndrome type 2 and short QT syndrome type 1.
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Protein Aliases: Eag homolog; eag-related protein 1; ERG-1; Ether-a-go-go-related gene potassium channel 1; ether-a-go-go-related potassium channel protein; ether-a-go-go-related protein 1; HERG-1; M-erg1; potassium channel, voltage gated eag related subfamily H, member 2; Potassium voltage-gated channel subfamily H member 2; potassium voltage-gated channel, subfamily H (eag-related), member 2; r-ERG; RERG; Voltage-gated potassium channel subunit Kv11.1
Gene Aliases: AI326795; ERG; ERG-1; ERG1; H-ERG; HERG; HERG1; KCNH2; Kv11.1; LQT; LQT2; M-erg; Merg1; merg1a; merg1b; SQT1
Molecular Function: ion channel