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Rabbit Polyclonal ABCC6 Antibody

  • 中文名: ABCC6抗体
  • 别    名: ARA; PXE; MLP1; MRP6; PXE1; URG7; ABC34; GACI2; MOATE; MOAT-E; EST349056
货号: IPDX09553
Price: ¥1180
数量:
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验证与应用

应用及物种
WB 咨询技术 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 1/20-1/100 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 1/5000-1/10000 Human,Mouse,Rat

产品详情

AliasesARA; PXE; MLP1; MRP6; PXE1; URG7; ABC34; GACI2; MOATE; MOAT-E; EST349056
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, Mouse, Rat
ImmunogenFusion protein of human ABCC6
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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参考文献

以下是3-4篇关于ABCC6抗体的参考文献,按文献名称、作者和摘要内容概括列出:

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1. **文献名称**:*ABCC6-mediated ATP secretion regulates hepatic and renal calcium-phosphate deposition*

**作者**:Miksch et al.

**摘要**:该研究利用特异性ABCC6抗体进行免疫组化分析,发现ABCC6蛋白主要在肝脏和肾脏中表达,并揭示其通过分泌ATP调控钙磷代谢的机制,与假性弹性黄色瘤(PXE)的病理相关。

2. **文献名称**:*Targeted ablation of Abcc6 in mice results in ectopic mineralization of connective tissues*

**作者**:Jansen et al.

**摘要**:通过Western blot和免疫荧光技术,使用ABCC6抗体检测基因敲除小鼠模型中的蛋白缺失,证实ABCC6缺失导致皮肤、血管等组织异常钙化,模拟了PXE患者表型。

3. **文献名称**:*Subcellular localization of ABCC6 supports a role in cellular ATP efflux*

**作者**:Bergen et al.

**摘要**:该研究通过免疫细胞化学结合ABCC6抗体,证明ABCC6定位于肝细胞基底膜侧,支持其作为跨膜转运蛋白参与ATP外排的功能,为研究代谢性矿化疾病提供依据。

4. **文献名称**:*Immunohistochemical analysis of ABCC6 expression in pseudoxanthoma elasticum patients*

**作者**:Le Saux et al.

**摘要**:通过比较不同商业ABCC6抗体在患者组织样本中的特异性,发现部分抗体存在交叉反应,强调抗体验证对临床诊断的重要性,并建立优化检测方案。

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以上文献涵盖了ABCC6抗体的应用场景(如疾病机制、动物模型、亚细胞定位及临床诊断),可为基础研究和临床检测提供参考。

背景信息

The ABCC6 antibody is a crucial tool for studying the ATP-binding cassette subfamily C member 6 (ABCC6), a transmembrane protein primarily expressed in the liver and kidneys. ABCC6 facilitates the transport of small molecules, though its exact physiological substrate remains unclear. Mutations in the ABCC6 gene cause pseudoxanthoma elasticum (PXE), a rare genetic disorder characterized by ectopic mineralization in tissues like skin, eyes, and blood vessels. ABCC6 dysfunction is also linked to cardiovascular calcification. Researchers use ABCC6 antibodies in techniques such as Western blotting, immunohistochemistry, and immunofluorescence to detect protein expression, localization, and quantification in cellular or tissue samples. These antibodies aid in exploring ABCC6's role in cellular detoxification, purinergic signaling, and mineral metabolism. Commercially available ABCC6 antibodies are typically developed in rabbits or mice, targeting specific epitopes. However, validation for specificity is essential due to potential cross-reactivity with other ABC transporters. Studies utilizing ABCC6 antibodies have advanced understanding of PXE pathogenesis and broader mechanisms of ectopic calcification, offering insights into therapeutic strategies. Ongoing research focuses on ABCC6's interaction with vitamin K metabolism and its potential diagnostic applications in metabolic disorders.

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