Voltage-gated K+ channels in the plasma membrane control the repolarization and the frequency of action potentials in neurons, muscles and other excitable cells. A specific K+ channel, comprised of an alpha subunit KCNQ1 and a beta subunit KCNE1, a small protein which spans the membrane only once, is predominantly expressed in the heart and in the cochlea, and is responsible for regulating the slow, depolarization-activated potassium current. Mutations in the genes encoding for KCNQ1 and KCNE1 lead to cardiac disease because they directly impair electrical signaling, and mutations in KCNQ4 are implicated in the onset of deafness. KCNQ proteins, including KCNQ1 and KCNQ4, characteristically contain six transmembrane domains and function as tetramers. KCNQ4 forms heteromeric channels with KCNQ3 and is expressed in several tissues, including the cochlea, where it is present in outer hair cells.
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Protein Aliases: ATFB1; ATFB3; FLJ26167; IKs producing slow voltage-gated potassium channel subunit alpha; IKs producing slow voltage-gated potassium channel subunit alpha KvLQT1; Jervell and Lange-Nielsen syndrome 1; JLNS1; KCNA8; KCNA9; KCNQ1; KCNQ1_HUMAN; kidney and cardiac voltage dependend K+ channel; KQT-like 1; Kv1.9; Kv7.1; KVLQT1; long (electrocardiographic) QT syndrome; LQT; LQT1; potassium channel, voltage gated KQT-like subfamily Q, member 1; potassium voltage-gated channel KQT-like subfamily member 1; Potassium voltage-gated channel subfamily KQT member 1; potassium voltage-gated channel, KQT-like subfamily, member 1; potassium voltage-gated channel, subfamily Q, member 1; RWS; slow delayed rectifier channel subunit; SQT2; Voltage-gated potassium channel subunit Kv7.1; Ward-Romano syndrome 1; WRS
Gene Aliases: ATFB1; ATFB3; AW559127; JLNS1; KCNA8; KCNA9; KCNQ1; Kv1.9; Kv7.1; KVLQT1; LQT; LQT1; RWS; SQT2; WRS