首页 / 产品 / 抗体 / 一抗

Rabbit Monoclonal ATP7b Antibody

  • 中文名： ATP7b抗体
  • 别    名： ATP7B; PWD; WC1; WD; WND; WND/140 kDa;;ATP7B
货号: IPDX18468
Price： ￥1280
数量：
大包装询价

验证与应用

应用及物种
WB 1/1000-1/2000 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 咨询技术 Human,Mouse,Rat
ICC 1/50-1/200 Human,Mouse,Rat
FCM 1/20-1/100 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliasesATP7B; PWD; WC1; WD; WND; WND/140 kDa;;ATP7B
WB Predicted band size157 kDa
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman
ImmunogenA synthesized peptide derived from human ATP7B
FormulationPurified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol.

+ +

参考文献

以下是3-4条关于ATP7B抗体的参考文献及其摘要概括：

---

1. **"Characterization of ATP7B antibodies in Wilson disease diagnosis"**

*Authors: Müller T. et al. (2003)*

**摘要**：研究评估了ATP7B特异性抗体在威尔逊病(Wilson disease)诊断中的应用，发现其可用于检测患者肝细胞中ATP7B蛋白的异常表达或缺失。

2. **"Subcellular localization of ATP7B in hepatocytes using monoclonal antibodies"**

*Authors: Huster D. et al. (2005)*

**摘要**：通过开发高特异性单克隆抗体，揭示了ATP7B蛋白在肝细胞中的亚细胞定位(如高尔基体和溶酶体)，并探讨其在铜转运中的动态变化。

3. **"ATP7B trafficking and function in copper metabolism: Insights from antibody-based imaging"**

*Authors: Polishchuk R. et al. (2014)*

**摘要**：利用免疫荧光和免疫电镜技术，结合ATP7B抗体，阐明了ATP7B在铜离子稳态中的动态运输机制及其突变导致的功能障碍。

4. **"Advances in Wilson disease biomarkers: Role of ATP7B antibody assays"**

*Authors: Ferenci P. & Cauza E. (2017)*

**摘要**：综述了ATP7B抗体检测技术的发展，及其在威尔逊病早期诊断和基因型-表型关联研究中的潜在价值。

---

注：以上文献为示例，实际引用时需核实具体文章信息及准确性。

背景信息

ATP7b antibodies are immunological tools used to study the copper-transporting ATPase ATP7B, a critical enzyme for copper homeostasis. ATP7B, primarily expressed in the liver, brain, and kidneys, facilitates copper excretion into bile and incorporates it into ceruloplasmin. Mutations in the ATP7B gene cause Wilson disease (WD), a rare autosomal recessive disorder characterized by toxic copper accumulation in tissues. ATP7b antibodies target specific epitopes of the ATP7B protein, enabling researchers to detect its expression, localization, and functional abnormalities in WD models or patient samples. These antibodies are widely used in immunohistochemistry, Western blotting, and immunofluorescence to assess ATP7B levels in liver biopsies or cellular studies, aiding in disease diagnosis and mechanistic research. Commercially available ATP7b antibodies are typically raised in rabbits or mice using peptide antigens corresponding to conserved regions of the human protein. However, challenges remain in standardizing antibody specificity due to ATP7B's structural complexity and homology with its paralog ATP7A. Recent advances in monoclonal antibody development aim to improve reproducibility in clinical diagnostics and preclinical studies exploring copper-chelation therapies or gene-editing interventions for WD.

客户数据及评论

折叠内容

大包装询价

×