Sixma J J, de Groot P G
Department of Haematology, University Hospital of Utrecht, The Netherlands.
Mayo Clin Proc. 1991 Jun;66(6):628-33. doi: 10.1016/s0025-6196(12)60523-0.
The role of von Willebrand factor (vWF) in mediating platelet adhesion has been established with the use of in vitro perfusion systems. vWF binds to the subendothelium, changes in conformation, and is then able to interact with glycoprotein Ib. vWF deposited in the subendothelium is responsible for up to 40% of normal adhesion. The action of vWF is seen at high shear rates. It also acts at low shear rates, but other factors can assume its role. Binding of vWF to the subendothelium is not through the established collagen binding sites but involves a new domain on the vWF molecule. Collagen VI has been implicated as the vessel wall component involved. Deposition of vWF in the subendothelium is determined by growth conditions and activation state of the endothelial cell. Subendothelial vWF in von Willebrand's disease may support platelet adhesion in situations in which plasma vWF of the same patient does not.
利用体外灌注系统已证实血管性血友病因子(vWF)在介导血小板黏附中的作用。vWF与内皮下层结合,构象发生变化,进而能够与糖蛋白Ib相互作用。沉积在内皮下层的vWF对正常黏附的贡献率高达40%。vWF的作用在高剪切速率下可见。它在低剪切速率下也起作用,但其他因素可发挥其作用。vWF与内皮下层的结合并非通过已确定的胶原结合位点,而是涉及vWF分子上的一个新结构域。已表明Ⅵ型胶原是涉及的血管壁成分。vWF在内皮下层的沉积取决于内皮细胞的生长条件和激活状态。血管性血友病患者内皮下的vWF在相同患者血浆vWF无法支持血小板黏附的情况下可能支持血小板黏附。