Overview
| 别名 | Renin receptor; ATPase H(+)-transporting lysosomal accessory protein 2; ATPase H(+)-transporting lysosomal-interacting protein 2; ER-localized type I transmembrane adapter; Embryonic liver differentiation factor 10; N14F; Renin/prorenin receptor; Vacuolar ATP synthase membrane sector-associated protein M8-9; ATP6M8-9; V-ATPase M8.9 subunit) [Cleaved into: Renin receptor N-terminal fragment; Renin receptor C-terminal fragment] |
| 基因名 | ATP6AP2 |
| UniProt ID | O75787 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 8K8-S1-W5 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 39 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:100-1:200 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | ATPase H+ transporting accessory protein 2 (ATP6AP2) is a type I transmembrane subunit of the vacuolar H+-ATPase (V-ATPase) that localizes to endosomes and lysosomes. It is essential for the assembly of the V-ATPase complex, which mediates the acidification of the endo-lysosomal system required for protein degradation and proper glycosylation. Additionally, its extracellular N-terminal domain functions as a receptor for renin and prorenin, facilitating the conversion of angiotensinogen to angiotensin I and activating ERK1/2 signaling. Pathogenic variants in ATP6AP2 are linked to X-linked parkinsonism with spasticity and X-linked congenital disorder of glycosylation type Iir. These conditions are characterized by neurodevelopmental delay, seizures, and movement disorders, resulting from impaired V-ATPase function and disrupted cellular homeostasis. |
检测原理