Potassium voltage-gated channel subfamily H member 5 (KCNH5)

Potassium voltage-gated channel subfamily H member 5 (KCNH5) is a pore-forming (alpha) subunit of a voltage-gated delayed rectifier potassium channel, also known as Kv10.2 or EAG2. It mediates outward-rectifying potassium currents activated by membrane depolarization, with slow activation kinetics and little or no inactivation. KCNH5 channels, widely expressed in the brain and other tissues, play crucial roles in regulating neuronal excitability, neurotransmitter and hormone release, cardiac repolarization, and cell volume. Pathogenic mutations in KCNH5 have been associated with early-onset epileptic encephalopathies, developmental delays, and autism spectrum disorder. Antiepileptic drugs, including valproic acid, lamotrigine, and others, are reported to control seizures in patients carrying KCNH5 mutations, though no specific modulators of KCNH5 are clinically available. Evidence also suggests KCNH5 may contribute to cancer biology, but its role in oncology requires further validation.

Other names

EAG2hEAG2Kv10.2H-EAG2ether-à-go-go 2voltage-gated delayed rectifier potassium channel KCNH5ether-a-go-go-related potassium channel 2

Enhancement or stabilization of neuronal potassium currents (indirectly, via symptom control in epilepsy) Inhibition of seizure propagation by modifying neuronal excitability (through non-specific AEDs)