vWF在相关疾病中的研究进展

Progress in Research of vWF in Related Diseases

血管性血友病因子(vWF)是一种黏附、多聚糖蛋白,分布在血浆、血小板和内皮下,在血小板黏附到内皮下的过程中起着重要作用。此外,作为载体蛋白,vWF可稳定凝血因子Ⅷ,并支持凝血因子的活化。研究发现,vWF分泌障碍、vWF多聚体组装或者蛋白水解异常都会导致vWF浓度降低,这些都是血管性血友病(vWD)的特征。相反,超大分子vWF导致的vWF降解破坏是血栓性血小板减少性紫癜(TTP)的一个重要因素。此外,vWF参与肝硬化、酒精性肝炎以及肝移植相关的肝病的发生与进展。除了这些疾病,vWF水平已经被证实是血栓形成的危险因素。最新研究发现,vWF可以调节血管产生、肿瘤细胞转移以及免疫系统,但其具体机理还有待进一步研究。

vWF has been shown to play a vital role in platelet adhesion, platelet binding to collagen and factor VIII protection. Recent studies have implicated vWF as a regulator of angiogenesis, smooth muscle cell proliferation, tumor cell metastasis and crosstalk in the immune system. On the contrary, impaired vWF degra- dation of ULvWF is a strong risk factor for TTP. Apart from these two diseases, vWF levels have been regarded as a thrombosis risk. Ongoing research efforts are aimed at gaining a better understanding of how vWF is regu- lated in disease conditions, particularly vascular - related diseases, and to determine its potential as a biomarker.