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Antibodies
Anti-ATP7B Antibody
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产品名称:
Anti-ATP7B Antibody
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简单介绍
Anti-ATP7B
Antibody
Anti-ATP7B Antibody
的详细介绍
Overview
Name:
Anti-ATP7B
Antibody
See all ATP7B primary antibodies
Description:
Rabbit polyclonal antibody to ATP7B
Specificity:
The antibody detects endogenous level of total ATP7B protein.
Applications:
WB, IHC, IF
Reactivity:
Human, Mouse, Rat
Immunogen:
Recombinant protein of human ATP7B.
Host:
Rabbit
Clonality:
Polyclonal
Conjugate:
Unconjugated
Purification:
Antibodies were purified by affinity purification using immunogen.
Concentration:
1.0mg / ml
Formulation:
Supplied at 1.0mg / mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Storage:
Store at -20?C
Target
Function:
Copper ion transmembrane transporter involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.
Tissue Specificity:
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.
Involvement in Disease:
Wilson disease: An autosomal recessive disorder of copper metabolism in which copper cannot be incorporated into ceruloplasmin in liver, and cannot be excreted from the liver into the bile. Copper accumulates in the liver and subsequently in the brain and kidney. The disease is characterized by neurologic manifestations and signs of cirrhosis.
Sequence Similarities:
Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily.
Post-Translational Modification:
Isoform 1 may be proteolytically cleaved at the N-terminus to produce the WND/140 kDa form.
Cellular Location:
Golgi apparatus > trans-Golgi network membrane. Late endosome.
Predominantly found in the trans-Golgi network (TGN). Localized in the trans-Golgi network under low copper conditions, redistributes to cytoplasmic vesicles when cells are exposed to elevated copper levels, and then recycles back to the trans-Golgi network when copper is removed (PubMed:10942420). Not redistributed to the plasma membrane in response to elevated copper levels.
Database Links:
Entrez Gene: 540?Human
Entrez Gene: 11979?Mouse
Entrez Gene: 24218?Rat
Omim: 606882?Human
SwissProt: P35670?Human
SwissProt: Q64446?Mouse
SwissProt: Q64535?Rat
Unigene: 492280?Human
Synonyms:
ATP7b Antibody
ATP7B_HUMAN Antibody
ATPase, Cu(2+) transporting, beta polypeptide Antibody
ATPase, Cu++ transporting, beta polypeptide Antibody
Copper pump 2 Antibody
Copper transporting ATPase 2 Antibody
PWD Antibody
Toxic milk Antibody
tx Antibody
WC1 Antibody
WD Antibody
Wilson disease associated protein Antibody
Wilson disease-associated protein Antibody
WND Antibody
WND/140 kDa Antibody
Information:
Target information shown above is from the UniProt Consortium.
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