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Anti-ATP7B Antibody

品牌：Antibodies

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規(guī)格：100μl

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Anti-ATP7B Antibody

商品詳情參考文獻相關(guān)資料

Overview

Name:	Anti-ATP7B Antibody See all ATP7B primary antibodies
Description:	Rabbit polyclonal antibody to ATP7B
Specificity:	The antibody detects endogenous levels of total ATP7B protein.
Applications:	IHC
Dilutions:	Immunohistochemistry: 1:20-1:100
Reactivity:	Human
Immunogen:	Synthetic peptide of human ATP7B
Host:	Rabbit
Clonality:	Polyclonal
Conjugate:	Unconjugated
Purification:	Antigen affinity purification.
Concentration:	1.4mg / ml
Formulation:	Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% 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 Omim: 606882?Human SwissProt: P35670?Human 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.