抗人 C1R 抗体对
01AB02939
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.
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技术参数
- 靶点基因名
- C1R
- 蛋白别名
- complement C1r; C1R
- UniProt
- P00736
- 物种
- Human
产品详情
- 靶点名称
- C1R
- 蛋白名称
- complement C1r
- 蛋白别名
- 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]
- 靶点基因名
- C1R
- UniProt
- P00736
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
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.