AAA Biotech, LLC's ATP7B Antibody is a Rabbit Polyclonal antibody. This antibody has been shown to work in applications such as: EIA, Immunoassay, ELISA, Immunocytochemistry, Immunofluorescence, and Immunohistochemistry. The ATP7B Antibody was generated using ATP7B as the antigen and it reacts with Homo Sapiens, Human, Mouse, and Rat.
Description
Description: The protein encoded by this gene belongs to the family of P-type cation transport ATPases. This gene encodes a polypeptide that acts as a membrane copper-transport protein. Defects in this gene are the cause of Wilson disease (WD). WD is an autosomal recessive disorder of copper metabolism in which copper cannot be incorporated into ceruloplasmin in liver, and cannot be excreted from the liver into the bile. Alternatively spliced transcript variants encoding different isoforms have been identified.
Function: Copper ion transmembrane transporter involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.
Subunit Structure: Monomer. Interacts with COMMD1/MURR1 (PubMed:12968035, PubMed:17919502). Interacts with DCTN4, in a copper-dependent manner (PubMed:16554302). Interacts with ATOX1 (PubMed:18558714). Interacts (via C-terminus) with ZBTB16/PLZF (PubMed:16676348).
Post-translational Modifications: Isoform 1 may be proteolytically cleaved at the N-terminus to produce the WND/140 kDa form.
Similarity: Each HMA domain can bind a copper ion, they are tightly packed and closely interact with each other. Wild-type ATP7B can usually be loaded with an average 5.5 copper atoms per molecule. Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily