MyBioSource.com's CLC2 (Clcn2, Chloride Channel) Antibody is a Rabbit Polyclonal antibody. This antibody has been shown to work in applications such as: EIA, Immunoassay, ELISA, Immunohistochemistry, and Western Blot. The CLC2 (Clcn2, Chloride Channel) Antibody was generated using CLCN2 as the antigen and it reacts with Rat.
Description
Chloride is a critical component of all living cells. Voltage-gated chloride channels regulate cellular traffic of chloride ions. The chloride channels (CIC or CLC) performs several functions including the regulation of cell volume, membrane potential stabilization, signal transduction, and transepithelial transport. Mutations in CIC genes have been linked with several human diseases including myotonias (Thomsen's disease), cystic fibrosis, Bartters syndrome type III, Dent's disease, and X-linked recessive nephrolithiasis. In mammals, CLC proteins form a superfamily of at least 9 different genes (CLC1-7 also known as CLCN1-7 and CLK1-2 or CLCKa and CLCKb). Additional forms of these proteins are obtained by alternative splicing. All CLC proteins (~700-1000aa) are predicted to contain 10 (possibly 12) transmembrane domains. Except CLC-1 and CLC-K1/K2 that are specific for kidney, most other CLC are widely distributed in various tissues. Rat CLC-2 is 887aa (or 907aa) membrane protein (human CLC-2 898aa) (1). An alternatively spliced short form (491aa) has also been found. CLC-2 is ubiquitously expressed. Defects in CLC1 (CLCN1) are the cause of autosomal recessive generalized myotonia (Becker's disease) (RGM) and autosomal dominant myotonia congenita (Thomsen's disease; MC) which are characterized by skeletal muscle stiffness (delayed relaxation) that is a result of muscle membrane hyperexcitability