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ATP7B, Antibody

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产品名称: ATP7B, Antibody
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简单介绍

ATP7B, Antibody


ATP7B, Antibody  的详细介绍
Product Name

ATP7B, Antibody

Full Product Name

Anti-ATP7B Antibody

Product Synonym Names
PWD; WC1; WD; WND
Product Gene Name

anti-ATP7B antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
277900
3D Structure
ModBase 3D Structure for P35670
Isotype
IgG
Host
Rabbit
Species Reactivity
Human
Purity/Purification
>=95% as determined by SDS-PAGE
Immunogen Affinity Purified
Form/Format
Liquid; PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
Immunogen
ATP7B
Preparation and Storage
Store at -20 degree C for 24 months. Avoid repeated freeze/thaw cycles.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
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.
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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
Immunohistochemistry (IHC)
Application Notes for anti-ATP7B antibody
IHC: 1:50-1:200
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NCBI/Uniprot data below describe general gene information for ATP7B. It may not necessarily be applicable to this product.
NCBI GI #
55743071
NCBI GeneID
540
NCBI Accession #
NP_000044.2 [Other Products]
NCBI GenBank Nucleotide #
NM_000053.3 [Other Products]
UniProt Primary Accession #
P35670 [Other Products]
UniProt Related Accession #
P35670[Other Products]
Molecular Weight
30 kDa
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NCBI Official Full Name
copper-transporting ATPase 2 isoform a
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]
UniProt Entry Name
ATP7B_HUMAN
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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]
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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: Transporter; Vesicle; Membrane protein, integral; Transporter, ion channel; Hydrolase; Membrane protein, multi-pass; EC 3.6.3.54

Chromosomal Location of Human Ortholog: 13q14.3

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

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

Biological Process: lactation; cellular copper ion homeostasis; response to copper ion; metabolic process; cellular zinc ion homeostasis; sequestering of calcium ion; copper ion import; copper ion transport; copper ion export; transmembrane transport; intracellular copper ion transport

Disease: Wilson Disease
Research Articles on ATP7B
1. A) located in the two different ATP7B alleles.">Study reported a patient with Wilson's disease (WD) presenting with a novel deletion mutation (c.532_574del43) and a known missense mutation (c.3517G > A) located in the two different ATP7B alleles.
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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