Protein Family Models

This family of ion channels contains 10 or 12 transmembrane helices. Each protein forms a single pore. It has been shown that some members of this family form homodimers. In terms of primary structure, they are unrelated to known cation channels or other types of anion channels. Three ClC subfamilies are found in animals. ClC-1 (Swiss:P35523) is involved in setting and restoring the resting membrane potential of skeletal muscle, while other channels play important parts in solute concentration mechanisms in the kidney [3]. These proteins contain two Pfam:PF00571 domains. [1]. 9252364. Reconstitution of functional voltage-gated chloride channels from complementary fragments of CLC-1. Schmidt-Rose T, Jentsch TJ;. J Biol Chem 1997;272:20515-20521. [2]. 8857733. Mutations in the human skeletal muscle chloride channel gene (CLCN1) associated with dominant and recessive myotonia congenita. Zhang J, George AL Jr, Griggs RC, Fouad GT, Roberts J, Kwiecinski H, Connolly AM, Ptacek LJ;. Neurology 1996;47:993-998. [3]. 11182894. ClC chloride channels. Mindell JA, Maduke M;. Genome Biol 2001;2:REVIEWS3003. (from Pfam)

Date:

2024-10-16

voltage-gated ClC-type chloride channel ClcB acts as an electrical shunt for an outwardly-directed proton pump that is linked to amino acid decarboxylation, as part of the extreme acid resistance (XAR) response

Date:

2019-06-06

Gene:

clcB

Date:

2021-09-22