KCNE3 Background Information Voltage-gated K+ channels in the plasma membrane control the repolarization and the frequency of action potentials in neurons, muscles, and other excitable cells. KCNE3 (potassium voltage-gated channel, Isk-related family, member 3), also known as HYPP, potassium channel subunit beta MiRP2 or HOKPP, is a 103 amino acid single-pass type I membrane protein belonging to the potassium channel KCNE family. Expressed predominantly in kidney, KCNE3 is also found at moderate levels in small intestine and associates with a voltage-gated potassium channel complex to regulate stability and gating kinetics. The gene encoding KCNE3 maps to human chromosome 11q13.4; defects in which are the cause of an autosomal dominant disorder known as periodic paralysis hypokalemic (or HOKPP), a muscular disorder known as thyrotoxic periodic paralysis type 1 (TTPP1) and Brugada syndrome type 6 (BRS6).
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