ATP7B; Copper pump 2; Coppertransporting ATPase 2; Copper-transporting ATPase 2; P35670; PWD; WND; WC1; Wilson disease-associated protein; ATPase, Cu++ transporting, beta polypeptide

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

Polyclonal

IgG

Rabbit

Immunogen affinity purified.

Lyophilized
Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.

Store at -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

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.

Rabbit IgG polyclonal antibody for Copper-transporting ATPase 2(ATP7B) detection.
Background: ATPase, Cu++ transporting, beta polypeptide (Wilson disease) protein, also called ATP7B, is an ATPase that transports copper. 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 two putative copper-s. ATP7B is mapped to 13q14.3. This protein functions as a monomer, exporting copper out of the cells. When copper levels are in excess, ATP7B redistributes to a vesicular compartment near the biliary canalicular membranes for elimination of excess copper into bile, and it is transported along liver cell microtubules via interaction with the p62 dynactin subunit.

Western Blot (WB), Immunohistochemistry (IHC) Paraffin

Western Blot: 0.1-0.5ug/ml
Immunohistochemistry(IHC) Paraffin: 0.5-1ug/ml

Western blot analysis of ATP7b expression in RH35 whole cell lysates (lane 1), HEPA whole cell lysates (lane 2) and HEPG2 whole cell lysates (lane 3). ATP7b at 157KD was detected using rabbit anti- ATP7b Antigen Affinity purified polyclonal antibody at 0.5ug/mL. The blot was developed using chemiluminescence (ECL) method.

ATP7b was detected in paraffin-embedded sections of rat brain tissues using rabbit anti- ATP7b Antigen Affinity purified polyclonal antibody at 1ug/mL. The immunohistochemical section was developed using SABC method.

ATP7b was detected in paraffin-embedded sections of rat brain tissues using rabbit anti- ATP7b Antigen Affinity purified polyclonal antibody at 1ug/mL. The immunohistochemical section was developed using SABC method.

ATP7b was detected in paraffin-embedded sections of human glioma tissues using rabbit anti- ATP7b Antigen Affinity purified polyclonal antibody at 1ug/mL. The immunohistochemical section was developed using SABC method.

155,125 Da

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]

ATP7B: Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile. Monomer. Interacts with COMMD1/MURR1. Most 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. Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 3.6.3.54; Hydrolase; Membrane protein, integral; Membrane protein, multi-pass; Transporter; Transporter, ion channel; Vesicle

Chromosomal Location of Human Ortholog: 13q14.3

Cellular Component: basolateral plasma membrane; cytoplasmic vesicle; Golgi membrane; integral to plasma membrane; late endosome; membrane; perinuclear region of cytoplasm; trans-Golgi network; trans-Golgi network membrane

Molecular Function: ATP binding; copper ion binding; copper ion transmembrane transporter activity; copper-exporting ATPase activity; copper-transporting ATPase activity; protein binding

Biological Process: cellular copper ion homeostasis; copper ion import; copper ion transport; response to copper ion; sequestering of calcium ion

Disease: Wilson Disease

1. "Entrez Gene: ATP7B ATPase, Cu++ transporting, beta polypeptide".
2. Lim, C. M., Cater, M. A., Mercer, J. F. B., La Fontaine, S. Copper-dependent interaction of dynactin subunit p62 with the N terminus of ATP7B but not ATP7A. J. Biol. Chem. 281: 14006-14014, 2006.

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