Ni H, Denis C V, Subbarao S, Degen J L, Sato T N, Hynes R O, Wagner D D
The Center for Blood Research, Boston, Massachusetts 02115, USA.
J Clin Invest. 2000 Aug;106(3):385-92. doi: 10.1172/JCI9896.
We used intravital microscopy to observe the formation of platelet plugs in ferric chloride-injured arterioles of live mice. With this model, we evaluated thrombus growth in mice lacking von Willebrand factor (vWF) and fibrinogen (Fg), the two key ligands known to mediate platelet adhesion and aggregation. In vWF(-/-) mice, despite the presence of arterial shear, delayed platelet adhesion occurred and stable thrombi formed. In many mice, a persisting high-shear channel never occluded. Abundant thrombi formed in Fg(-/-) mice, but they detached from the subendothelium, which ultimately caused downstream occlusion in all cases. Surprisingly, mice deficient in both vWF and Fg successfully formed thrombi with properties characteristic of both mutations, leading to vessel occlusion in the majority of vessels. Platelets of these doubly deficient mice specifically accumulated fibronectin in their alpha-granules, suggesting that fibronectin could be the ligand supporting the platelet aggregation.
我们采用活体显微镜观察了活小鼠经氯化铁损伤的小动脉中血小板栓子的形成。利用该模型,我们评估了缺乏血管性血友病因子(vWF)和纤维蛋白原(Fg)的小鼠体内血栓的生长情况,这两种关键配体已知可介导血小板黏附和聚集。在vWF基因敲除(-/-)小鼠中,尽管存在动脉切变力,但血小板黏附延迟,且形成了稳定的血栓。在许多小鼠中,持续存在的高切变通道从未闭塞。Fg基因敲除(-/-)小鼠中形成了大量血栓,但它们从内皮下脱离,最终在所有情况下都导致了下游血管闭塞。令人惊讶的是,同时缺乏vWF和Fg的小鼠成功形成了具有两种突变特征的血栓,导致大多数血管发生闭塞。这些双基因敲除小鼠的血小板在其α颗粒中特异性积累纤连蛋白,表明纤连蛋白可能是支持血小板聚集的配体。