FXIIa 和血浆激肽释放酶活性的调节
中文名称
通路描述
激活的因子 XIIa 和血浆激肽释放酶通过协调炎症信号通路、补体激活、凝血和纤溶来发挥作用（参考 Renne T 等人，2012; Maas C & Renne T, 2018; Schmaier AH, 2016）。该系统受到多种蛋白质的严格调控，包括血浆蛋白酶 C1 抑制剂（C1INH，由 SERPING1 基因编码）、α2-巨球蛋白（由 A2M 基因编码）和肝素结合糖蛋白（HRG），这些蛋白质防止接触激活系统和血浆激肽 - 激肽系统的过度激活，并保护免受病理血栓和炎症条件的影响（Pixley RA 等人，1985; Harpel PC 等人，1985; MacQuarrie JL 等人，2011）。C1INH 和α2-巨球蛋白是血浆激肽释放酶的主要血浆蛋白酶抑制剂，分别占 42% 和 48% 的抑制作用（Schapira M 等人，1981），尽管不同研究中的相对贡献有所不同（Schapira M 等人，1981; Harpel 等人，1985）。C1INH（SERPING1）是 FXIIa 的主要抑制剂，占血浆 FXIIa 抑制的 92%（Pixley RA 等人，1985）。
英文描述
Regulation of FXIIa and plasma kallikrein activity Activated factor XIIa and plasma kallikrein coordinate inflammatory signaling pathways, complement activation, coagulation, and fibrinolysis (reviewed by Renne T et al., 2012; Maas C & Renne T, 2018; Schmaier AH, 2016). This system is tightly regulated by several proteins, including plasma protease C1 inhibitor (C1INH, encoded by the SERPING1 gene), alpha-2-macroglobulin (encoded by the A2M gene), and heparin-binding glycoprotein (HRG), which prevent overactivation of contact activation system and plasma kallikrein-kinin system and protect against pathological thrombotic and inflammatory conditions (Pixley RA et al., 1985; Harpel PC et al., 1985; MacQuarrie JL et al., 2011). C1INH and alpha-2-macroglobulin are the major plasma protease inhibitors of plasma kallikrein accounting for 42 and 48% inhibition, respectively (Schapira M et al., 1981), although their relative contributions vary among studies (Schapira M et al., 1981; Harpel et al., 1985). C1INH (SERPING1) is the predominant inhibitor of FXIIa, accounting for 92% plasma FXIIa inhibition (Pixley RA et al., 1985).
所含基因
8 个基因