CHEMICAL AND STRUCTURAL STUDIES ON FACTOR VIII/VON WILLEBRAND FACTOR PROTEIN

本项目的长期目标最初是研究 因子VIII/von Willebrand因子的免疫学方面 蛋白质，然后研究它的生物化学和结构， 目前感兴趣的主题是：1）缺陷 血友病Ap中的因子VIII/血管性血友病因子蛋白; 2)第八因子/血管性血友病因子蛋白的缺陷， 血管性血友病; 3）碳水化合物的重要性 碳水化合物与生物功能的关系 5）凝血酶的作用机制 活化;和6）生物学特性的生物化学表征 因子VIII/von Willebrand因子蛋白的活性位点。 最近研究的血小板血管性血友病因子分离自 人血小板显示出与其血浆的显著差异 对应物。 研究更好的纯化方法将使 更高的产量，这反过来又将允许表征 血小板血管性血友病因子

The long range purpose of this project initially is to study the immunologic aspects of the factor VIII/von Willebrand factor protein, and then to study the biochemistry and structure of this protein in man. The topics of present interest are: 1) the defect of the factor VIII/von Willebrand factor protein in hemophilia Ap; 2) the defect of the factor VIII/von Willebrand factor protein in von Willebrand's disease; 3) the importance of carbohydrate content in biological functions; 4) the relationship of carbohydrate content to atherosclerosis; 5) the mechanism of thrombin activation; and 6) biochemical characterization of the biologically active sites of the factor VIII/von Willebrand factor protein. Recent studies of the platelet von Willebrand factor isolated from human platelets reveal significant differences from its plasma counterpart. Studies of better purification methods will allow for greater yields which, in turn, will allow characterization of the platelet von Willebrand factor.