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AB217299

Anti-ATP7b 抗体 - C-terminal

Anti-ATP7b antibody - C-terminal

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(1 Publication)

Rabbit Polyclonal ATP7B antibody. C-terminal. Suitable for IHC-P and reacts with Rat samples. Cited in 1 publication. Immunogen corresponding to Synthetic Peptide within Human ATP7B aa 1400 to C-terminus conjugated to Keyhole Limpet Haemocyanin.

別名を表示する

PWD, WC1, WND, ATP7B, Copper-transporting ATPase 2, Copper pump 2, Wilson disease-associated protein

1 Images
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-ATP7b antibody - C-terminal (AB217299)
  • IHC-P

Supplier Data

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-ATP7b antibody - C-terminal (AB217299)

Immunohistochemical analysis of formalin-fixed and paraffin-embedded rat brain tissue labeling ATP7b with ab217299 at 1/200 dilution, followed by conjugation to the secondary antibody and DAB staining.

Key facts

宿主種

Rabbit

クローン性

Polyclonal

アイソタイプ

IgG

キャリアフリー

No

交差種

Rat

アプリケーション

IHC-P

applications

免疫原

Synthetic Peptide within Human ATP7B aa 1400 to C-terminus conjugated to Keyhole Limpet Haemocyanin. The exact immunogen used to generate this antibody is proprietary information.

P35670

Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "IHCP-species-checked": "predicted", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" }, "Mouse": { "IHCP-species-checked": "predicted", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" }, "Rat": { "IHCP-species-checked": "testedAndGuaranteed", "IHCP-species-dilution-info": "1/100 - 1/500", "IHCP-species-notes": "<p></p>" } } }

出荷温度及び保存条件

製品の状態
Liquid
精製方法
Affinity purification Protein A
バッファー組成
pH: 7.4 Preservative: 0.02% Proclin 300 Constituents: 50% Glycerol (glycerin, glycerine), 48.98% TBS, 1X, 1% BSA
出荷温度
Blue Ice
短期保存期間
1-2 weeks
短期保存温度
+4°C
長期保存温度
-20°C
分注に関する情報
Upon delivery aliquot
保管に関する情報
Avoid freeze / thaw cycle

補足情報

This supplementary information is collated from multiple sources and compiled automatically.

ATP7B also known as Wilson disease protein is a copper-transporting ATPase. This protein has a mass of approximately 146 kDa. It is mainly expressed in the liver kidney placenta and brain. ATP7B functions mechanically by transporting copper across different cellular compartments. It utilizes ATP hydrolysis to pump copper ions helping the body regulate copper homeostasis. This transport activity is located predominantly in the trans-Golgi network where ATP7B assists in incorporating copper into ceruloplasmin a critical copper-carrying blood plasma protein.
Biological function summary

ATP7B plays a significant role in maintaining copper balance within the body. It associates with intracellular vesicles and through its catalytic activity influences various cellular metabolic processes. Although ATP7B does not typically form large complexes its interaction with other proteins and cellular organelles contributes to copper ion binding and transfer. Proper ATP7B function ensures that copper is channeled effectively to places where it is required for enzymatic activity or is expelled from cells to prevent accumulation.

Pathways

ATP7B is a central figure in the copper transport and homeostasis pathway. Its role is directly connected to the biosynthesis of copper-dependent enzymes. The protein impacts the pathway involving ceruloplasmin biosynthesis by regulating copper ion incorporation. ATP7A another copper-transporting ATPase shares similar pathway responsibilities but in different tissues showing divergence in their specific biological roles. Together they ensure whole-body copper balance.

ATP7B mutations or dysfunction is strongly implicated in Wilson's disease a genetic disorder characterized by excessive copper accumulation. This can result in hepatic neurological and psychiatric symptoms due to copper buildup. The protein's relationship to Wilson's disease suggests its critical role in preventing copper toxicity. There is also evidence linking ATP7B with Menkes disease but ATP7A plays a more direct role in Menkes highlighting the distinct responsibilities these ATPases have in different tissues and conditions.

製品プロトコール

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ターゲットの情報

Copper ion transmembrane transporter involved in the export of copper out of the cells. It is involved in copper homeostasis in the liver, where it ensures the efflux of copper from hepatocytes into the bile in response to copper overload.
See full target information ATP7B

文献 (1)

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Applications

Unspecified application

Species

Unspecified reactive species

Wenbo Liu,Xukun Lu,Zheng-Hui Zhao,Ruibao Su,Qian-Nan Li Li,Yue Xue,Zheng Gao,Si-Min Sun Sun,Wen-Long Lei,Lei Li,Geng An,Hanyan Liu,Zhiming Han,Ying-Chun Ouyang,Yi Hou,Zhen-Bo Wang,Qing-Yuan Sun,Jianqiao Liu
View all publications

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