ATP7b / Unconjugated /

Product Details
Supplier Aviva Systems Biology
Catalog #: OALA05854 (View supplier product page)
Size 50 μg
Price $395.00
Antigen ATP7b
Clone
Host Rabbit
Isotype IgG
Conjugate Unconjugated
Target Species Human, Mouse, Rat
Applications IHC-P, IF, ELISA
Description ATP7b (ATPase, Cu++ Transporting, Beta Polypeptide), also known as Wilson Disease gene (WND) or mouse toxic milk gene, plays a key role in copper transport. Germ-line mutations in this gene cause so-called Wilson disease, which is characterized by the accumulation of toxic levels of copper in the liver, brain, and kidney resulting in extensive tissue damage and eventual death. ATP7b is expressed in a variety of carcinomas and appears to be associated with chemoresistance to cisplatin.
About ATP7b and Purified
ATP7b This gene is a member of the P-type cation transport ATPase family and encodes a protein with several membrane-spanning domains, an ATPase consensus sequence, a hinge domain, a phosphorylation site, and at least 2 putative copper-binding sites. This protein is a monomer, and functions as a copper-transporting ATPase which exports copper out of the cells, such as the efflux of hepatic copper into the bile. Alternate transcriptional splice variants, encoding different isoforms with distinct cellular localizations, have been characterized. Mutations in this gene have been associated with Wilson disease which is characterized by copper accumulation. [provided by RefSeq, Dec 2019]
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Purified Excitation and Emission Spectra