Overview
| 别名 | Kin of IRRE-like protein 1; Kin of irregular chiasm-like protein 1; Nephrin-like protein 1 |
| 基因名 | KIRREL1 |
| UniProt ID | Q96J84 |
| 反应种属 | Human |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 8Z1-F4-J3 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 83 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000 |
| 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 | Kirre like nephrin family adhesion molecule 1 (KIRREL1) is a transmembrane adhesion molecule primarily expressed in kidney podocytes, where it is essential for maintaining the integrity of the glomerular filtration barrier. It features extracellular immunoglobulin-like domains that facilitate cell-cell adhesion, forming the specialized slit diaphragm junctions between podocyte foot processes. The cytoplasmic domain of KIRREL1 interacts with podocin and signaling molecules like TEC kinases to activate downstream transcription factors, ensuring size- and charge-selective ultrafiltration. Mutations in KIRREL1 are well-established causes of Nephrotic Syndrome Type 23, a rare hereditary disorder characterized by childhood-onset steroid-resistant proteinuria, edema, and progression to end-stage renal disease. |
检测原理