Hoyer L W
Prog Clin Biol Res. 1981;72:1-26.
Normal human plasma contains a complex of two proteins that are important in hemostasis and coagulation. The factor VIII procoagulant protein (antihemophilic factor) and the factor VIII-related protein (von Willebrand factor) are under separate genetic control, have distinct biochemical properties, and have unique and essential physiologic properties. While the nature of their interaction and the details of their biochemical structure remain to be determined, the information now available permits a preliminary understanding of the molecular defects in the factor VIII deficiency disease.
正常人血浆含有两种在止血和凝血过程中起重要作用的蛋白质复合物。凝血因子VIII促凝血蛋白(抗血友病因子)和凝血因子VIII相关蛋白(血管性血友病因子)受不同的基因控制,具有不同的生化特性,且具有独特且必不可少的生理特性。虽然它们相互作用的性质及其生化结构的细节仍有待确定,但目前可获得的信息有助于初步了解凝血因子VIII缺乏症中的分子缺陷。