ATP7B, Polyclonal Antibody

ATP7B; ATP7B_HUMAN; ATPase, Cu(2+) transporting, beta polypeptide; ATPase, Cu++ transporting, beta polypeptide; Copper pump 2; Copper transporting ATPase 2; PWD; Toxic milk; tx; WC1; WD; Wilson disease associated protein; Wilson disease-associated protein; WND; WND/140 kDa

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

ATP7B antibody detects endogenous levels of ATP7B

The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin.

Liquid
Phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

1mg/ml (lot specific)

Store at -20 degree C. Stable for 12 months from date of receipt.

Manufactured in an ISO 9001:2015 Certified Laboratory.

Manufactured in a lab with traceable raw materials manufactured on site. Coordinated product portfolio of antibodies, pairs, conjugates, recombinant proteins, and immunoassay materials available, please inquire.

Small volumes of anti-ATP7B antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Immunohistochemisty (IHC), Immunofluorescence (IF), Immunocytochemistry (ICC), ELISA (EIA)

IHC: 1:50-1:200
IF/ICC: 1:100-1:500

MBS9600320 at 1/100 staining human colon tissue sections by IHC-P. The tissue was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. The tissue was then blocked and incubated with the antibody for 1.5 hours at 22 degree C. An HRP conjugated goat anti-rabbit antibody was used as the secondary.

MBS9600320 staining CACO-2 cells by IF/ICC. The sample were fixed with PFA and permeabilized in 0.1% Triton X-100, then blocked in 10% serum for 45 minutes at 25 degree C. The primary antibody was diluted at 1/200 and incubated with the sample for 1 hour at 37 degree C. An Alexa Fluor 594 conjugated goat anti-rabbit IgG (H+L) antibody, diluted at 1/600, was used as secondary antibody.

Observed: 157 kDa
Predicted: 158 kDa

ATPase copper transporting beta

copper-transporting ATPase 2

Copper-transporting ATPase 2

PWD; WC1; WND  [Similar Products]

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-s. This protein functions as a monomer, exporting 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 (WD). [provided by RefSeq, Jul 2008]

Copper ion transmembrane transporter involved in the export of copper out of the cells. It is involved in copper homeostasis in the liver, where it ensures the efflux of copper from hepatocytes into the bile in response to copper overload.

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