Stel H V, van der Kwast T H, Veerman E C
Nature. 1983;303(5917):530-2. doi: 10.1038/303530a0.
Factor VIII, a high molecular weight glycoprotein complex which has an important role in haemostasis, consists of two immunologically as well as functionally discernible moieties that can be isolated separately. These are factor VIII/von Willebrand factor (FVIII/vWF) which is associated with the factor VIII-related antigen (FVIIIRAg), and the factor VIII/procoagulant activity (FVIII/C) which is associated with the factor VIII/procoagulant antigen (FVIII/CAg). The FVIII/C activity is decreased or absent in patients with haemophilia A (for a review of the structure and function of the factor VIII complex, see refs 1 and 2). Immunological techniques, combined with cell culture, have demonstrated that FVIIIRAg is present in and synthesized by endothelial cells and megakaryocytes. However, the organ and/or cell type responsible for the production of FVIII/C has not been established. Indirect evidence derived from organ transplantation in experimental animals suggests that the liver is the most likely organ for FVIII/C production. Here we have used a monoclonal antibody against FVIII/CAg in combination with a sensitive immunostaining technique to demonstrate the presence of FVIII/CAg in hepatic sinusoidal endothelial cells.
凝血因子VIII是一种在止血过程中起重要作用的高分子量糖蛋白复合物，由两个在免疫学和功能上均可区分的部分组成，这两个部分可以分别分离出来。它们是与凝血因子VIII相关抗原（FVIIIRAg）相关的凝血因子VIII/血管性血友病因子（FVIII/vWF），以及与凝血因子VIII促凝血活性（FVIII/C）相关的凝血因子VIII促凝血抗原（FVIII/CAg）。甲型血友病患者的FVIII/C活性降低或缺乏（有关凝血因子VIII复合物的结构和功能综述，请参阅参考文献1和2）。免疫技术与细胞培养相结合已证明，FVIIIRAg存在于内皮细胞和巨核细胞中并由它们合成。然而，负责产生FVIII/C的器官和/或细胞类型尚未确定。来自实验动物器官移植的间接证据表明，肝脏是产生FVIII/C最可能的器官。在此，我们使用了一种针对FVIII/CAg的单克隆抗体，结合敏感的免疫染色技术，来证明肝窦内皮细胞中存在FVIII/CAg。
