Gralnick H R, Coller B S, Sultan Y
Science. 1976 Apr 2;192(4234):56-9. doi: 10.1126/science.1083071.
Study of the normal human factor VIII/von Willebrand factor reveals a macromolecular glycoprotein composed of apparently identical subunits. This purified glycoprotein has procoagulant, antigen, and von Willebrand factor activities. In three patients with a variant of the von Willebrand's disease syndrome, their factor VIII/von Willebrand factor protein was present in normal amounts and had normal procoagulant and antigen activities; however, this protein was deficient in both carbohydrate and von Willebrand factor activity. The carbohydrate portion of the factor VIII/von Willebrand factor glycoprotein is of major importance in its interactions with platelets or the blood vessel wall, or both.
对正常人因子VIII/血管性血友病因子的研究表明，它是一种由明显相同的亚基组成的大分子糖蛋白。这种纯化的糖蛋白具有促凝血、抗原和血管性血友病因子活性。在三名患有血管性血友病综合征变体的患者中，他们的因子VIII/血管性血友病因子蛋白含量正常，具有正常的促凝血和抗原活性；然而，这种蛋白在碳水化合物和血管性血友病因子活性方面均有缺陷。因子VIII/血管性血友病因子糖蛋白的碳水化合物部分在其与血小板或血管壁或两者的相互作用中至关重要。
