Recombinant Human ATP7B
Cat.No. : | ATP7B-174H |
Product Overview : | Recombinant Wilson Protein (ATP7B, Copper-transporting ATPase 2, Copper pump 2, Wilson disease-associated protein) was cloned from human cDNA with a N-terminal purification tag and expressed inE.coli. The protein consists of the all six soluble domains of the human Wilson protein (residues 1-636 swissprot accession P35670) and contains 3 additional aa (SFT) in the apoform. N-term end of the protein. MW = 67.8 KDa. |
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- Gene Information
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Cat. No. : | ATP7B-174H |
Description : | 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-binding sites. This protein functions as a monomer, exporting copper out of the cells, such as the efflux of hepatic copper into the bile. |
Source : | E.Coli. |
Purity : | >90% by SDS-PAGE. The protein was observed as a single band migrating at molecular weight of 66 kDa. |
Supplied As : | 1mg/ml in sodium phosphate buffer 50mM pH=7.0 , arginine 50mM, glutammic acid 50mM, DTT 2mM, EDTA 1mM, pefabloc® 50uM. The concentration is calculated from the absorbance at 280nm (ε280= 24410 M-1cm-1). |
Characteriatias : | To avoid precipitation handle the protein in an inert atmosphere, the product can be concentrated to a maximum of 0.3mM. |
Usage : | metal binding studies, kinetics, WB, SDS-page, blotting, ELISA, antibody production. |
Storage : | -20ºC. The protein is stable at 25ºC for at least several hours. After initial defrost, aliquot product into individual tubes and refreeze at -20ºC. Avoid repeated freeze/defrost cycles. |
Gene Name : | ATP7B ATPase, Cu++ transporting, beta polypeptide [ Homo sapiens ] |
Synonyms : | ATP7B; ATPase,Cu++ transporting, beta polypeptide; WD; PWD; WC1; WND; ATP7B; ATPase, Cu++ transporting, beta polypeptide (Wilson disease); Wilson disease;Copper-transporting ATPase 2;EC3.6.3.4; Copper pump 2; Wilson; disease-associated protein; wn/140KDa; OTTHUMP00000040880; ATPase, Cu(2+)- transporting, beta polypeptide; ATPase, Cu++ transporting, beta polypeptide (Wilson disease); Wilson disease-associated protein |
Gene ID : | 540 |
mRNA Refseq : | NM_000053 |
Protein Refseq : | NP_000044 |
MIM : | 606882 |
UniProt ID : | P35670 |
Chromosome Location : | 13q14.3 |
Function : | ATP binding;copper ion binding;copper-exporting ATPase activity;copper-exporting ATPase activity;hydrolase activity;hydrolase activity, acting on acid anhydrides, catalyzing transmembrane;movement of substances;magnesium ion binding;metal ion transmembrane transporter activity;nucleotide binding;protein binding |
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For Research Use Only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.
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Q&As (5)
Ask a questionATP7B facilitates copper transport from the cytoplasm to the Golgi apparatus, where copper is incorporated into proteins. It also mediates copper export from cells when copper levels are elevated, preventing toxicity.
ATP7B interacts with various proteins in the copper transport pathway. Disruptions in these interactions can lead to mislocalization of ATP7B and compromise its ability to regulate copper levels properly.
ATP7B ensures the proper supply of copper to enzymes involved in various cellular processes, including energy production and antioxidant defense. Dysregulation can disrupt these processes, affecting cell function.
ATP7B is crucial for copper excretion into bile, a process that contributes to systemic copper balance. Dysfunctional ATP7B results in impaired copper excretion, leading to the accumulation of copper in tissues.
Mutations in the ATP7B gene lead to impaired copper transport, causing copper accumulation in tissues. This is associated with Wilson's disease, a disorder characterized by copper toxicity, especially in the liver and brain.
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Write a reviewImpressed with the product quality.
The product exceeded expectations.
This product consistently delivers accurate results in our experiments.
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