| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/25-1/100 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/2000-1/5000 | Human,Mouse,Rat |
| Aliases | WD; PWD; WC1; WND |
| 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 | Synthetic peptide of human ATP7B |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是3-4条关于ATP7B抗体的参考文献示例(内容为模拟概括，非真实文献)：
1. **《ATP7B Antibody Characterization in Wilson Disease Diagnosis》**
- 作者：Schushan, B. et al.
- 摘要：研究报道了一种新型ATP7B多克隆抗体的开发，验证其在肝细胞中ATP7B蛋白定位检测的敏感性，并探讨其在威尔逊病(Wilson Disease)患者肝组织病理学诊断中的应用价值。
2. **《Epitope Mapping of ATP7B Autoantibodies in Autoimmune Liver Disorders》**
- 作者：Dijkstra, M.K. & Müller, T.
- 摘要：通过免疫印迹和肽库筛选技术，揭示了ATP7B抗体在自身免疫性肝炎(AIH)患者中的表位特异性，提出其可能与威尔逊病重叠的免疫病理机制。
3. **《ATP7B Antibodies as Biomarkers for Copper Metabolism Dysregulation》**
- 作者：Huster, D. & Weiss, K.H.
- 摘要：分析了ATP7B抗体水平与血清铜蓝蛋白及尿铜排泄的关联性，表明其在监测铜代谢异常疾病中的潜在辅助诊断作用。
4. **《Comparative Study of Commercial ATP7B Antibodies in Immunohistochemistry》**
- 作者：Cauza, E. et al.
- 摘要：系统比较了多种商业化ATP7B抗体在肝细胞切片中的染色效果，为临床和科研中选择高特异性抗体提供参考依据。
(注：以上文献名为虚构，摘要内容为领域典型研究方向概括，实际文献需通过PubMed/Google Scholar等平台检索。)
ATP7B antibody is a crucial tool in studying Wilson disease, a rare autosomal recessive disorder caused by mutations in the ATP7B gene. The ATP7B protein, a copper-transporting P-type ATPase, plays a vital role in copper homeostasis by facilitating its excretion into bile and incorporation into ceruloplasmin. Expressed predominantly in the liver, ATP7B maintains systemic copper balance by trafficking between the trans-Golgi network and cytoplasmic vesicles under varying copper levels. Mutations in ATP7B disrupt copper excretion, leading to toxic copper accumulation in the liver, brain, and other organs, resulting in Wilson disease's clinical manifestations, including liver cirrhosis and neurological deficits.
Antibodies targeting ATP7B are widely used in research and diagnostics to detect protein expression, localization, and functional abnormalities. In research, these antibodies enable techniques like Western blotting, immunohistochemistry, and immunofluorescence to study ATP7B's role in cellular copper regulation and disease mechanisms. Clinically, they assist in confirming Wilson disease diagnoses by identifying reduced or mislocalized ATP7B in liver biopsies. Additionally, ATP7B antibodies contribute to therapeutic development, such as evaluating gene therapy efficacy or small-molecule interventions aimed at restoring copper transport. Their specificity and reliability make them indispensable for advancing understanding and management of copper metabolism disorders.
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