Ruggeri Zaverio M
The Scripps Research Institute, La Jolla, California, USA.
Thromb Res. 2007;120 Suppl 1(Suppl 1):S5-9. doi: 10.1016/j.thromres.2007.03.011. Epub 2007 May 9.
Von Willebrand factor (VWF) is a large multimeric glycoprotein produced in endothelial cells and megakaryocytes and present in subendothelial matrix, blood plasma and platelets. VWF mediates adhesion and aggregation of platelets at sites of vascular injury, processes that are critical for both haemostasis and thrombosis. Thrombus formation involves complex events that are influenced by different environmental conditions. Progress in understanding the structure and function of VWF and the mechanisms that underlie its interactions with platelets has led to important insight into the differentiation between normal haemostasis and pathological arterial thrombosis. The conventional view of signalling-induced platelet aggregation has recently been extended to include activation-independent aggregation. A novel mechanism has been demonstrated for initiating thrombus formation under high haemodynamic forces that involves alpha(IIb)beta(3)-independent platelet aggregation at the interface between immobilised and soluble VWF. This VWF-mediated process may be a key determinant of platelet accumulation in stenotic arteries leading to acute thrombotic occlusion.
血管性血友病因子（VWF）是一种由内皮细胞和巨核细胞产生的大型多聚体糖蛋白，存在于内皮下基质、血浆和血小板中。VWF介导血管损伤部位血小板的黏附和聚集，这些过程对于止血和血栓形成都至关重要。血栓形成涉及受不同环境条件影响的复杂事件。在理解VWF的结构和功能以及其与血小板相互作用的机制方面取得的进展，为深入了解正常止血与病理性动脉血栓形成之间的差异提供了重要见解。信号诱导的血小板聚集的传统观点最近已扩展到包括非激活依赖性聚集。已经证明了一种在高血流动力学力下启动血栓形成的新机制，该机制涉及在固定化和可溶性VWF之间的界面处发生的不依赖α（IIb）β（3）的血小板聚集。这种VWF介导的过程可能是导致急性血栓闭塞的狭窄动脉中血小板聚集的关键决定因素。
