凝血级联的调节
中文名称
通路描述
凝血途径通过多种机制紧密调节，以维持止血平衡并确保有效凝血。任何成分的失调都可能导致出血或血栓性疾病。此 Reactome 模块描述了凝血途径的关键内源性调节因子，包括：组织因子途径抑制物（TFPI）：与 FXa、FVIIa 和 TF 形成四聚体复合物，有效地抑制 FXa 和 FVIIa，限制启动阶段的凝血（Broze GJ et al., 1990; Maroney SA et al., 2013）。蛋白 C：由完整内皮细胞上的凝血酶 - 凝血模体复合物激活。与其辅因子蛋白 S 一起，它抑制 FVa 和 FVIIIa 活性，有助于抗凝血（Stavenuiter F et al., 2013）。丝氨酸内肽酶抑制剂，包括 SERPINC1（抗凝血酶 III）、SERPINA5、SERPINA10 和 SERPIND1：通过不可逆地结合其丝氨酸激活位点，抑制丝氨酸蛋白酶如凝血酶和 FXa（Mushunje A et al., 2003; Boulaftali Y et al., 2010; Huang X et al., 2010）。13 型整合素 - 金属蛋白酶 - 纤连蛋白结构域（ADAMTS13）：一种金属蛋白酶，在因子 VIII 存在下，在血小板表面或循环中切割 VWF 多聚体，调节血栓形成（Cao W et al., 2008; DeYoung V et al., 2022）。此 Reactome 模块还强调了靶向凝血因子或其调节剂以促进有效凝血形成的选定药物（Petersen LC 2012; Nutescu EA et al., 2016）。
英文描述
Fibrin formation This Reactome module describes the multi-step process of fibrin formation, which is initiated by the thrombin (FIIa)-catalyzed conversion of fibrinogen molecules to fibrin monomers (Ni F et al., 1989; Pechik I et al., 2006; Riedel T et al., 2011). Fibrin monomers rapidly and spontaneously associate into large multimers, which in turn elongate into protofibrils that later aggregate to form fibers, creating a three-dimensional network. This structural organization is essential for the mechanical properties of the clot (reviewed by Doolittle 1984; Litvinov RI & Weisel JW 2017; Feller T et al., 2022). The fibrin network is further stabilized by Ca2+ -dependent cross-linking of fibers, which is mediated by the thrombin-activated factor XIII (FXIIIa). Adhesion of integrin αIIbβ3, a platelet surface receptor, to the fibrin network contributes to platelet aggregation and stabilizes the forming clot (Litvinov RI et al., 2016; Höök P et al., 2017). The process of fibrin clot formation has been reviewed in detail (Medved L & Weisel JW 2009; Weisel JW & Litvinov RI 2017; Pieters M& Wolberg AS 2019; Butera D & Hogg PJ 2020; Murano G 2024).
所含基因
13 个基因