Schreiber A D
Semin Thromb Hemost. 1976 Jul;3(1):32-51.
The Hageman factor dependent pathways are influenced by several control proteins which modulate the extent of activation and biologic activity of these enzyme substrates (Fig. 1). C1 INH plays a prominent role by acting at the common initiating step for all three Hageman factor dependent systems and its deficiency produces disease in man. Alpha-2 macroglobulin appears to play an important role in the fibrinolytic sequence, having potent activity towards both plasminogen activator and plasmin. Antithrombin most prominently influences the state of activation of the coagulation sequence by regulating the enzymatic activities of activated Factors XI, IX and X and, most importantly, that of thrombin. Significantly, deficiency of antithrombin results in increased thrombosis in man.
接触因子依赖性途径受几种调控蛋白的影响，这些调控蛋白可调节这些酶底物的激活程度和生物学活性（图1）。C1抑制因子通过作用于所有三种接触因子依赖性系统的共同起始步骤发挥重要作用，其缺乏会在人类中引发疾病。α2巨球蛋白似乎在纤维蛋白溶解序列中发挥重要作用，对纤溶酶原激活剂和纤溶酶均具有强大活性。抗凝血酶通过调节活化的因子Ⅺ、Ⅸ和Ⅹ的酶活性，最重要的是调节凝血酶的酶活性，最显著地影响凝血序列的激活状态。值得注意的是，抗凝血酶缺乏会导致人类血栓形成增加。
