Overview
| 别名 | Complement C1r subcomponent; Complement component 1 subcomponent r) [Cleaved into: Complement C1r subcomponent heavy chain; Complement C1r subcomponent chain A); Complement C1r subcomponent light chain; Complement C1r subcomponent chain B] |
| 基因名 | C1R |
| UniProt ID | P00736 |
| 反应种属 | Human |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 4A7-I4-Z4 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 80 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; IHC-1:200-1:250 |
| 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 | Complement C1r (C1R) is a secreted calcium-binding serine protease that serves as a catalytic component of the C1 complex, the initiator of the classical complement pathway. Existing as a homodimer within the C1q-C1r2-C1s2 complex, C1r is autoactivated upon C1q binding to immune complexes. It subsequently cleaves and activates C1s, triggering the downstream complement cascade essential for innate immunity. Beyond immune defense, C1r interacts with extracellular matrix components. Specific pathogenic variants in the C1R gene cause periodontal Ehlers-Danlos syndrome, an autosomal dominant connective tissue disorder characterized by severe periodontitis and tissue fragility, resulting from aberrant protease activity and matrix destabilization. |
检测原理