Perutelli P, Mori P G
Recenti Prog Med. 1997 Nov;88(11):526-9.
The human von Willebrand factor (vWf) is a multimeric glycoprotein present in plasma, platelets, endothelial cells and subendothelium and synthesized in endothelial cells and megakaryocytes. vWf plays a pivotal role in the mechanisms of blood clotting and platelet thrombus formation; quantitative and qualitative abnormalities of vWf cause the most common congenital bleeding disorder in man, the von Willebrand disease. vWf stabilizes factor VIII and interacts with subendothelial components and with platelet membrane receptors. The multimeric structure of vWf provides an array of binding sites which allows multivalent interactions with its ligands, thus supporting the formation of stable platelet aggregates at the site of vascular injury, particularly under flow conditions characterized by high shear stress. In the last years, remarkable progress has been made toward understanding the structure of vWf protein and gene, and the elucidation of many structure-function relationships, which may result in improved therapeutic intervention for vWD patients, and in the development of effective strategies for antithrombotic therapy.
人血管性血友病因子（vWf）是一种存在于血浆、血小板、内皮细胞和内皮下层的多聚体糖蛋白，由内皮细胞和巨核细胞合成。vWf在血液凝固和血小板血栓形成机制中起关键作用；vWf的数量和质量异常会导致人类最常见的先天性出血性疾病——血管性血友病。vWf可稳定凝血因子VIII，并与内皮下成分及血小板膜受体相互作用。vWf的多聚体结构提供了一系列结合位点，使其能够与其配体进行多价相互作用，从而在血管损伤部位，特别是在高剪切应力的流动条件下，支持稳定的血小板聚集体的形成。近年来，在理解vWf蛋白和基因的结构以及阐明许多结构-功能关系方面取得了显著进展，这可能会改善对血管性血友病患者的治疗干预，并开发出有效的抗血栓治疗策略。
