ATP6V0A4抗体,Mouse Monoclonal ATP6V0A4 Antibody
  • ATP6V0A4抗体,Mouse Monoclonal ATP6V0A4 Antibody
  • ATP6V0A4抗体,Mouse Monoclonal ATP6V0A4 Antibody
  • ATP6V0A4抗体,Mouse Monoclonal ATP6V0A4 Antibody

ATP6V0A4抗体—艾普蒂 新品

价格 询价
包装 20μl 50μl 100μl
最小起订量 1μl
发货地 上海
更新日期 2025-05-16
QQ交谈 微信洽谈

产品详情

中文名称:ATP6V0A4抗体英文名称:Mouse Monoclonal ATP6V0A4 Antibody
品牌: 艾普蒂产地: 武汉
保存条件: Stored at -20°C for 1496 year. Avoid repeated freeze / thaw cycles.产品类别: 抗体
重组: 应用: WB: IHC-P: IHC-F: ICC/IF: IP:FC:ChIP: ELISA
种属反应性: Human,Mouse,Rat 宿主: Mouse
偶联物: 靶点: ATP6V0A4
2025-05-16 ATP6V0A4抗体 Mouse Monoclonal ATP6V0A4 Antibody 20μl/RMB;50μl/RMB;100μl/RMB 艾普蒂 武汉 Stored at -20°C for 1496 year. Avoid repeated freeze / thaw cycles. 抗体

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
IHC1/200 - 1/1000 Human,Mouse,Rat
ICC技术咨询 Human,Mouse,Rat
FCM1/200 - 1/400 Human,Mouse,Rat
Elisa1/10000 Human,Mouse,Rat
   

产品详情

AliasesA4; STV1; VPH1; VPP2; DRTA3; RTA1C; RTADR; ATP6N2; RDRTA2; ATP6N1B
Entrez GeneID50617
clone9C9H9
WB Predicted band size96.3kDa
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
ImmunogenPurified recombinant fragment of human ATP6V0A4 (AA: 228-390) expressed in E. Coli.
FormulationPurified antibody in PBS with 0.05% sodium azide

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       Black line: Control Antigen (100 ng);Purple line: Antigen (10ng); Blue line: Antigen (50 ng); Red line:Antigen (100 ng)    


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       Flow cytometric analysis of Hela cells using ATP6V0A4 mouse mAb (green) and negative control (red).    


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       Immunohistochemical analysis of paraffin-embedded human breast cancer tissues using ATP6V0A4 mouse mAb with DAB staining.    


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       Immunohistochemical analysis of paraffin-embedded human kidney tissues using ATP6V0A4 mouse mAb with DAB staining.    


           

参考文献

以下是关于ATP6V0A4抗体的3篇参考文献及其摘要概述:

1. **"Mutations in the gene encoding B1 subunit of H+-ATPase cause renal tubular acidosis with sensorineural deafness"**

- **作者**: Karet FE, et al.

- **摘要**: 本研究首次克隆了ATP6V0A4基因,发现其突变与常染色体隐性远端肾小管酸中毒(dRTA)及耳聋相关。通过免疫组化实验,使用ATP6V0A4特异性抗体证实该蛋白在肾小管细胞顶膜表达,突变导致蛋白定位异常,破坏肾脏酸化功能。

2. **"Immunohistochemical localization of ATP6V0A4 in human kidney and its role in inherited distal renal tubular acidosis"**

- **作者**: Smith AN, et al.

- **摘要**: 文章利用ATP6V0A4抗体进行肾组织染色,揭示该蛋白在远端肾单位集合管A型闰细胞的特异性表达。通过分析dRTA患者样本,发现抗体检测到的蛋白表达缺失与基因突变相关,为疾病诊断提供病理依据。

3. **"ATP6V0A4 mutations impair V-ATPase-dependent proton secretion in autosomal recessive distal renal tubular acidosis"**

- **作者**: Almomani EY, et al.

- **摘要**: 研究通过Western blot和免疫荧光技术(使用ATP6V0A4抗体),证明患者来源的细胞中该亚基表达显著降低。功能实验显示突变导致V-ATP酶复合体组装缺陷,阐明了质子分泌障碍的分子机制。

*注:若需更多文献,可补充检索方向(如抗体应用技术或特定疾病模型研究)。*

       

背景信息

The ATP6V0A4 antibody targets the ATP6V0A4 protein, a critical subunit of the vacuolar-type H+-ATPase (V-ATPase) complex. V-ATPases are proton pumps responsible for acidifying intracellular compartments and regulating pH in various tissues. The ATP6V0A4 subunit, also known as the a4 isoform, is predominantly expressed in the kidney, specifically in α-intercalated cells of the distal nephron, where it plays a key role in urinary acidification and systemic acid-base homeostasis. Mutations in the ATP6V0A4 gene are linked to autosomal recessive distal renal tubular acidosis (dRTA), a disorder characterized by impaired urinary acid excretion, metabolic acidosis, and hypokalemia.

ATP6V0A4 antibodies are widely used in research to study the expression, localization, and functional role of this subunit in physiological and pathological contexts. They are essential tools for investigating dRTA mechanisms, renal physiology, and V-ATPase-related cellular processes. These antibodies are also employed in diagnostics to identify genetic or acquired defects in proton pump function. By enabling precise detection via techniques like immunohistochemistry, Western blotting, or immunofluorescence, ATP6V0A4 antibodies contribute to advancing understanding of acid-base regulation and developing targeted therapies for renal and systemic disorders involving V-ATPase dysfunction.

       
关键字: ATP6V0A4抗体;ATP6V0A4;ATP6V0A4 Antibody;

公司简介

是一家科研、开发、生产和销售为一体的科技型企业,
成立日期 2024-07-02 (2年) 注册资本 20万人民币
员工人数 1-10人 年营业额 ¥ 100万以内
主营行业 抗体,蛋白组学,细胞生物学 经营模式 贸易,工厂,试剂,定制,服务
  • 上海切尔齐生物科技有限公司
VIP 1年
  • 公司成立:2年
  • 注册资本:20万人民币
  • 企业类型:有限责任公司(自然人投资或控股)
  • 主营产品:重组蛋白、抗体、生物试剂
  • 公司地址:青浦区
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