Guillin M C, Bezeaud A
Pathol Biol (Paris). 1985 Nov;33(9):917-32.
Inopportune coagulation of blood in vessels is prevented by defense mechanisms, in which plasma inhibitors play an important role. The inhibitors are glycoproteins and belong to two different groups, according to their mechanism of action. The first group consists of the inhibitors of serine proteases, which form inactive complexes with various coagulation enzymes; it includes antithrombin III, heparin cofactor II, alpha 2-macroglobulin, alpha 1-antitrypsin and C1S-inhibitor. The second group includes protein C and its cofactor, protein S. Protein C, activated by thrombin complexed with a protein cofactor present on the endothelial cell surface (thrombomodulin), is responsible for the proteolytic degradation of two coagulation cofactors (Va and VIII: Ca). The clinical importance of both antithrombin III, protein C and protein S is attested by the strong association between recurrent venous thromboembolic manifestations and inherited deficiencies of one or the other of these proteins.
血管内血液的不当凝固可通过防御机制来预防，其中血浆抑制剂起着重要作用。根据其作用机制，这些抑制剂是糖蛋白，可分为两个不同的组。第一组由丝氨酸蛋白酶抑制剂组成，它们与各种凝血酶形成无活性复合物；包括抗凝血酶III、肝素辅因子II、α2-巨球蛋白、α1-抗胰蛋白酶和C1S抑制剂。第二组包括蛋白C及其辅因子蛋白S。蛋白C由与内皮细胞表面存在的蛋白辅因子（血栓调节蛋白）结合的凝血酶激活，负责两种凝血辅因子（Va和VIII：Ca）的蛋白水解降解。抗凝血酶III、蛋白C和蛋白S的临床重要性通过复发性静脉血栓栓塞表现与这些蛋白中一种或另一种的遗传性缺乏之间的强烈关联得到证实。
