| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Copper-transporting ATPase 2, Copper pump 2, Wilson disease-associated protein, WND/140 kDa, ATP7B, PWD, WC1, WND |
| Entrez GeneID | 540 |
| WB Predicted band size | 157.3kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human, Mouse, Rat |
| Immunogen | This ATP7B antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 1361-1391 amino acids from the C-terminal region of human ATP7B. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,1%BSA and 50% glycerol.prepared by Saturated Ammonium Sulfate (SAS) . |
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以下是关于ATP7B抗体的3篇代表性文献示例(文献信息为模拟概括，供参考)：
1. **标题**: *ATP7B antibody-based detection of Wilson disease-associated protein variants*
**作者**: Suzuki M, et al.
**摘要**: 研究开发了一种高特异性ATP7B多克隆抗体，用于检测威尔逊病患者肝细胞中ATP7B蛋白的异常定位及表达水平下降，验证了抗体在免疫组化诊断中的应用价值。
2. **标题**: *A novel ELISA for quantification of ATP7B protein in human serum*
**作者**: Weiss KH, et al.
**摘要**: 提出基于ATP7B抗体的ELISA检测方法，用于定量血清中ATP7B蛋白片段浓度，发现威尔逊病患者血清ATP7B水平显著低于健康对照组，提示其作为潜在生物标志物的可能性。
3. **标题**: *Copper-induced trafficking of ATP7B in hepatocytes: Insights from immunofluorescence studies*
**作者**: Polishchuk R, et al.
**摘要**: 利用ATP7B特异性单克隆抗体，通过免疫荧光技术揭示铜离子浓度变化对ATP7B蛋白在肝细胞中亚细胞定位的影响，阐明了其铜转运功能的动态调控机制。
注：以上内容为基于领域知识的模拟概括，实际文献需通过PubMed/Google Scholar等平台以“ATP7B antibody”或“ATP7B immunohistochemistry”等关键词检索。建议优先参考近5年《Journal of Hepatology》《Metallomics》等期刊的相关论文。
ATP7B antibody is a crucial tool in studying Wilson's disease, a rare autosomal recessive disorder caused by mutations in the ATP7B gene. The ATP7B protein, a copper-transporting P-type ATPase, regulates copper homeostasis by facilitating its excretion into bile and incorporation into ceruloplasmin. Mutations in ATP7B disrupt copper metabolism, leading to toxic accumulation in the liver, brain, and other organs.
ATP7B antibodies are specifically designed to detect and quantify the ATP7B protein in research and diagnostic settings. They are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to assess ATP7B expression, localization, and function in cellular or tissue samples. In Wilson's disease research, these antibodies help identify protein truncations, misfolding, or trafficking defects caused by pathogenic mutations. Commercially available ATP7B antibodies target distinct epitopes, such as the N-terminal copper-binding domain or transmembrane regions, enabling diverse experimental applications.
Clinically, ATP7B antibodies may aid in confirming Wilson's disease diagnosis when genetic testing is inconclusive, though their utility is currently secondary to biochemical and molecular analyses. Recent studies also explore ATP7B's role beyond copper metabolism, including cancer progression and neurodegenerative disorders, broadening the antibody's research relevance. Validation of antibody specificity remains critical due to potential cross-reactivity with homologous proteins like ATP7A.
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