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C1-抑制物(C1-INH)是含478个氨基酸的单链糖蛋白,为补体经典激活途径主要调节因子。C1-INH与靶酶相互作用时,反应中心关键的P_1和P_1’位点为Arg_(444)-Thr~(445)。C1-INH遗传性和获得性缺乏可引起血管神经性水肿,其分子发病机制各不相同。其它多种疾病包括系统性红斑狼疮(SLE),遗传性C_4缺乏症等也与C1-INH缺乏或功能障碍有关。IFN-γ,TNF,IL-6,M-CSF,糖皮质激素有增强C1-INH基因表达的作用。
The C1-inhibitor (C1-INH) is a 478 amino acid single-chain glycoprotein that is the major regulator of the classical complement activation pathway. When C1-INH interacts with the target enzyme, the key P 1 and P 1 ’sites in the reaction center are Arg 444 -Thr 445. C1-INH Hereditary and acquired deficiency can cause vascular neurogenic edema, its molecular pathogenesis varies. A variety of other diseases, including systemic lupus erythematosus (SLE), hereditary C_4 deficiency, etc., are also associated with a lack of C1-INH or dysfunction. IFN-γ, TNF, IL-6, M-CSF and glucocorticoids can enhance the expression of C1-INH gene.