Kv1.1

Potassium voltage-gated channel subfamily A member 1 also known as K_v1.1 is a shaker related voltage-gated potassium channel that in humans is encoded by the KCNA1 gene. The Isaacs syndrome is a result of an autoimmune reaction against the K_v1.1 ion channel.

Genomics

The gene is located on the Watson (plus) strand of the short arm of chromosome 12 (12p13.32). The gene itself is 8,348 bases in length and encodes a protein of 495 amino acids (predicted molecular weight 56.466 kiloDaltons).

Alternative names

The recommended name for this protein is potassium voltage-gated channel subfamily A member 1 but a number of alternatives have been used in the literature including HuK1 (human K⁺ channel I), RBK1 (rubidium potassium channel 1), MBK (mouse brain K⁺ channel), voltage gated potassium channel HBK1, voltage gated potassium channel subunit K_v1.1, voltage-gated K⁺ channel HuKI and AEMK (associated with myokymia with periodic ataxia).

Structure

The protein is believed to have six domains (S1-S6) with the loop between S5 and S6 forming the channel pore. This region also has a conserved selectivity filter motif. The functional channel is a homotetramer. The N-terminus of the protein associates with β subunits. These subunits regulate channel inactivation as well as its expression. The C-terminus is associated with a PDZ domain protein involved in channel targeting.