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ATPase, Cu++ Transporting Beta Polypeptide (ATP7b), Polyclonal Antibody

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产品名称: ATPase, Cu++ Transporting Beta Polypeptide (ATP7b), Polyclonal Antibody
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简单介绍

ATPase, Cu++ Transporting Beta Polypeptide (ATP7b), Polyclonal Antibody


ATPase, Cu++ Transporting Beta Polypeptide (ATP7b), Polyclonal Antibody  的详细介绍
Product Name

ATPase, Cu++ Transporting Beta Polypeptide (ATP7b), Polyclonal Antibody

Full Product Name

PE-Linked Polyclonal Antibody to ATPase, Cu++ Transporting Beta Polypeptide (ATP7b)

Product Synonym Names
PWD; WC1; WD; WND; Wilson Disease Protein; Copper pump 2; Wilson disease-associated protein
Product Gene Name

anti-ATP7b antibody

[Similar Products]
Matching Pairs
Unconjugated Antibody: ATPase (MBS2028503)
PE Conjugated Antibody: ATPase, Cu++ Transporting Beta Polypeptide (ATP7b) (MBS2077981)
Matching Pairs
PE Conjugated Antibody: ATPase, Cu++ Transporting Beta Polypeptide (ATP7b) (MBS2077981)
Immunogen: ATPase (MBS2030977)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
U11700 mRNA
Clonality
Polyclonal
Host
Rabbit
Species Reactivity
Human
Concentration
200ug/ml (lot specific)
Immunogen
ATP7b (Thr59~Val426)
Conjugation
PE
Unconjugated Antibody
The unconjugated antibody version of this item is also available as catalog #MBS2028503
ISO Certification
Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.
Supply Chain Verification
Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customer’s specifications, please inquire.
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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Applications Tested/Suitable for anti-ATP7b antibody
Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (ELISA)
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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 #
62088502
NCBI GeneID
540
NCBI Accession #
BAD92698.1 [Other Products]
UniProt Secondary Accession #
Q16318; Q16319; Q4U3V3; Q59FJ9; Q5T7X7[Other Products]
UniProt Related Accession #
P35670[Other Products]
Molecular Weight
155,125 Da
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NCBI Official Full Name
ATPase, Cu++ transporting, beta polypeptide isoform a variant, 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]
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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: 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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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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