Matsushita T, Dong Z, Sadler J E
Howard Hughes Medical Institute, Washington University, St. Louis, Missouri, USA.
Curr Opin Hematol. 1994 Sep;1(5):362-8.
von Willebrand's factor is required for platelet adhesion to subendothelium, and for normal factor VIII survival in the circulation. These functions require the assembly of von Willebrand's factor into multimers that exhibit properly regulated binding to platelet glycoprotein lb. Recent studies suggest that the propeptide of von Willebrand's factor may catalyze multimer assembly and have identified new segments of von Willebrand's factor that appear to regulate its affinity for glycoprotein lb. Two segments of von Willebrand's factor have been found to interact with collagen type VI, which is a candidate binding site for von Willebrand's factor in the subendothelium. Advances in the identification of mutations have prompted a reclassification of von Willebrand's disease. ABO antigens on von Willebrand's factor may impair the efficacy of plasma or recombinant von Willebrand's factor when administered to patients with incompatible ABO blood type.
血管性血友病因子是血小板黏附于内皮下以及循环中正常因子Ⅷ存活所必需的。这些功能需要血管性血友病因子组装成多聚体，这些多聚体与血小板糖蛋白lb具有适当调节的结合能力。最近的研究表明，血管性血友病因子的前肽可能催化多聚体组装，并已确定了血管性血友病因子的新片段，这些片段似乎调节其对糖蛋白lb的亲和力。已发现血管性血友病因子的两个片段与Ⅵ型胶原相互作用，Ⅵ型胶原是血管性血友病因子在内皮下的一个候选结合位点。突变鉴定方面的进展促使对血管性血友病进行重新分类。当给血型不相容的血管性血友病患者使用血管性血友病因子时，血管性血友病因子上的ABO抗原可能会损害血浆或重组血管性血友病因子的疗效。
