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Mouse Monoclonal ATP2C1 Antibody

  • 中文名: ATP2C1抗体
  • 别    名: HHD; BCPM; PMR1; SPCA1
货号: IPD30001
Price: ¥1280
数量:
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验证与应用

应用及物种
WB 1/500 - 1/2000 Human,Monkey
IF 咨询技术 Human,Monkey
IHC 1/200 - 1/1000 Human,Monkey
ICC 技术咨询 Human,Monkey
FCM 咨询技术 Human,Monkey
Elisa 1/10000 Human,Monkey

产品详情

AliasesHHD; BCPM; PMR1; SPCA1
Entrez GeneID27032
clone4G12
WB Predicted band size100kDa
Host/IsotypeMouse IgG1
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman,Monkey
ImmunogenPurified recombinant fragment of ATP2C1 expressed in E. Coli.
FormulationAscitic fluid containing 0.03% sodium azide.

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参考文献

以下是关于ATP2C1抗体的3篇文献摘要信息:

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1. **文献名称**:*ATP2C1 mutations in Hailey-Hailey disease: A hotspot for molecular alterations*

**作者**:Hu Z, Bonifas JM, et al.

**摘要**:该研究通过基因测序鉴定了Hailey-Hailey病(HHD)患者中的ATP2C1基因突变,并利用ATP2C1特异性抗体进行Western blot分析,发现突变导致皮肤组织中SPCA1蛋白表达显著下降,揭示了该蛋白缺陷与疾病发生的关联。

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2. **文献名称**:*Localization and function of SPCA1 (ATP2C1) in the secretory pathway*

**作者**:Micaroni M, Perinetti G, et al.

**摘要**:研究使用ATP2C1抗体进行免疫荧光染色,证明SPCA1蛋白主要定位于高尔基体,参与钙离子转运和细胞分泌过程。抗体特异性验证表明,ATP2C1在维持高尔基体钙稳态中起关键作用。

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3. **文献名称**:*Development of a monoclonal antibody against human ATP2C1 for diagnostic applications*

**作者**:Suzuki Y, Sakisaka T, et al.

**摘要**:本文报道了一种新型抗人ATP2C1单克隆抗体的开发与验证,通过免疫组化技术证实该抗体可特异性识别皮肤和上皮组织中的SPCA1蛋白,为Hailey-Hailey病的临床诊断提供了可靠工具。

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以上文献均聚焦于ATP2C1抗体的应用,涵盖疾病机制、蛋白功能及诊断技术研究。

背景信息

ATP2C1 antibody targets the ATPase Secretory Pathway Ca²⁺ Transport 1 (SPCA1), encoded by the ATP2C1 gene. This protein belongs to the P-type ATPase family and regulates intracellular calcium and manganese ion homeostasis by actively transporting these ions into the Golgi apparatus. SPCA1 is critical for maintaining luminal calcium levels required for protein processing, trafficking, and signaling in secretory pathways. Dysregulation of ATP2C1 is linked to Hailey-Hailey disease, an autosomal-dominant skin disorder characterized by acantholysis (loss of cell adhesion) due to ATP2C1 mutations. ATP2C1 antibodies are essential tools in studying SPCA1 expression, localization, and function in both normal and diseased tissues. They are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to investigate SPCA1's role in epithelial integrity, calcium signaling, and stress responses. Research also explores ATP2C1's potential involvement in cancers (e.g., breast, colorectal) and neurodegenerative diseases, where altered calcium/manganese metabolism may contribute to pathogenesis. These antibodies aid in identifying ATP2C1 deficiency or overexpression, offering insights into disease mechanisms and therapeutic targets. Commercial ATP2C1 antibodies are typically validated for specificity across species, including human and mouse models.

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