Full Product Name
Anti-ATP7B Antibody
Product Gene Name
anti-ATP7B antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse, Rat
Specificity
Western blot analysis of extracts of various cell lines, using ATP7B antibody. Immunofluorescence analysis of U20S cell using ATP7B antibody. Blue: DAPI for nuclear staining.
Purity/Purification
Affinity Purification
Concentration
1mg/ml (lot specific)
Immunogen
Recombinant protein of human ATP7B
Storage Buffer
PBS with 0.02% sodium azide,50% glycerol, pH7.3.
Preparation and Storage
Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.
Other Notes
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.
Related Product Information for
anti-ATP7B antibody
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).
Applications Tested/Suitable for anti-ATP7B antibody
Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
Application Notes for anti-ATP7B antibody
WB: 1:500 - 1:2000
IHC: 1:50- 1:200
IF: 1:50- 1:200
Western Blot (WB) of anti-ATP7B antibody
Immunofluorescence (IF) of anti-ATP7B antibody
NCBI/Uniprot data below describe general gene information for ATP7B. It may not necessarily be applicable to this product.
NCBI Accession #
AAX84215.1
[Other Products]
UniProt Secondary Accession #
Q16318; Q16319; Q4U3V3; Q59FJ9; Q5T7X7[Other Products]
UniProt Related Accession #
P35670[Other Products]
Molecular Weight
155,125 Da
NCBI Official Full Name
ATP7B, partial
NCBI Official Synonym Full Names
ATPase copper transporting beta
NCBI Official Symbol
ATP7B??[Similar Products]
NCBI Official Synonym Symbols
WD; PWD; WC1; WND
??[Similar Products]
NCBI Protein Information
copper-transporting ATPase 2
UniProt Protein Name
Copper-transporting ATPase 2
UniProt Synonym Protein Names
Copper pump 2; Wilson disease-associated protein
UniProt Gene Name
ATP7B??[Similar Products]
UniProt Synonym Gene Names
PWD; WC1; WND??[Similar Products]
NCBI Summary for 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-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]
UniProt Comments for ATP7B
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
Research Articles on ATP7B
1. In the group of 75 Wilson Disease patients of Croatian origin, 18 different mutations in ATP7B gene were detected, three of which were novel. The p.His1069Gln mutation was most frequent, being detected in 44 Croatian WD patients (58.7%). Most ATP7B mutations (90.4%) were located in exons 5, 8, 13, 14, and 15.
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