Abstract—

Von Willebrand factor (vWF), the key component of hemostasis, is synthesized in endothelial cells and megakaryocytes and released into the blood as high molecular weight multimeric glycoproteins weighing up to 20 million Daltons. Blood plasma metalloprotease ADAMTS13 cleaves ultra-large vWF multimers to smaller multimeric and oligomeric molecules. The vWF molecules attach to the sites of damage at the surface of arterioles and capillaries and unfold under conditions of shear stress. On the unfolded vWF molecule, the regions interacting with receptors on the platelet membrane are exposed. After binding to the vWF filaments, platelets are activated; platelets circulating in the vessels are additionally attached to them, leading to thrombus formation, blocking of microvessels, and cessation of bleeding. This review describes the history of the discovery of vWF, presents data on the mechanisms of vWF secretion and its structure, and characterizes the processes of vWF metabolism in the body under normal and pathological conditions.

中文翻译：

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健康与疾病中的 Von Willebrand 因子

摘要——

血管性血友病因子 (vWF) 是止血的关键成分，在内皮细胞和巨核细胞中合成，并以重达 2000 万道尔顿的高分子量多聚体糖蛋白形式释放到血液中。血浆金属蛋白酶 ADAMTS13 将超大 vWF 多聚体切割成较小的多聚体和寡聚体分子。vWF 分子附着在小动脉和毛细血管表面的损伤部位，并在剪切应力条件下展开。在未折叠的 vWF 分子上，与血小板膜上受体相互作用的区域是暴露的。与 vWF 细丝结合后，血小板被激活；在血管中循环的血小板也附着在它们上面，导致血栓形成、微血管阻塞和出血停止。