Potassium voltage-gated channel subfamily H member 1 (KCNH1)

Potassium voltage-gated channel subfamily H member 1 (KCNH1, also called Kv10.1 or EAG1) is a tetrameric, non-inactivating delayed rectifier potassium channel predominantly expressed in the adult central nervous system[1][4]. It plays a critical role in restoring the membrane potential of excitatory neurons during periods of high-frequency firing, and its slow activation and lack of inactivation distinguish it from other voltage-gated potassium channels[1][3]. KCNH1 is also implicated in regulating cell proliferation and differentiation, especially in neuronal and myogenic contexts[1][3][4]. Pathogenic mutations cause developmental epileptic encephalopathies such as Temple-Baraitser and Zimmermann-Laband syndromes[1][3][4]. Overexpression of KCNH1 is a feature of several malignancies, where it can contribute to increased tumor cell proliferation[1][3][4]. KCNH1 is a potential anticancer drug target, and certain existing drugs (like astemizole and imipramine) block its activity, although these are not selective agents[1][4]. Safety concerns mirror those for other potassium channel inhibitors, particularly the risk of cardiac arrhythmia[3][4].

Other names

EAGEAG1hEAGhEAG1h-eageagKv10.1K(V)10.1Ether-a-go-go potassium channel 1Ether-a-go-go 1Voltage-gated potassium channel subunit Kv10.1Voltage-gated delayed rectifier potassium channel KCNH1Potassium voltage-gated channel, subfamily H (Eag-related), member 1TMBTSZLS1

Channel blockade (suppresses potassium ion flow to alter membrane potential); Inhibition of potassium conductance (suppresses excitability in neurons, or cell proliferation in tumor cells)