Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 40% Glycerol, PBS
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab262897 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||Use a concentration of 1 - 4 µg/ml.
Fixation/Permeabilization: PFA/Triton X-100
FunctionInvolved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.
Tissue specificityMost abundant in liver and kidney and also found in brain. Isoform 2 is expressed in brain but not in liver. The cleaved form WND/140 kDa is found in liver cell lines and other tissues.
Involvement in diseaseDefects in ATP7B are the cause of Wilson disease (WD) [MIM:277900]. 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. Copper accumulates in the liver and subsequently in the brain and kidney. The disease is characterized by neurologic manifestations and signs of cirrhosis.
Sequence similaritiesBelongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily.
Contains 6 HMA domains.
modificationsIsoform 1 may be proteolytically cleaved at the N-terminus to produce the WND/140 kDa form.
Cellular localizationCytoplasm; Mitochondrion and Golgi apparatus > trans-Golgi network membrane. Predominantly found in the trans-Golgi network (TGN). Not redistributed to the plasma membrane in response to elevated copper levels.
- Information by UniProt
- ATP7B antibody
- ATP7B_HUMAN antibody
- ATPase, Cu(2+) transporting, beta polypeptide antibody
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab262897 has not yet been referenced specifically in any publications.