von Willebrand factor is a cofactor in complement regulation
von Willebrand factor is a cofactor in complement regulation
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DOI：
10.1182/blood-2014-06-585430
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发表时间：
2015-02-05
期刊：
影响因子：
20.3
通讯作者：
Afshar-Kharghan, Vahid
中科院分区：
文献类型：
--
作者：
Feng, Shuju;Liang, Xiaowen;Afshar-Kharghan, Vahid
几种补体蛋白与止血因子相互作用。我们发现血管性血友病因子（VWF）作为因子i介导的补体C3b切割的辅助因子，从而关闭补体激活。VWF多定时器的补体调节功能取决于其大小。较小的VWF多聚体增强了C3b的切割，而较大和超大VWF （ULVWF）多聚体对C3b的切割没有影响，并允许默认补体激活。我们得出结论，正常血浆VWF多聚体阻止补体激活并引导补体途径产生失活的C3b （iC3b）。ULVWF多聚体，存在于血栓性微血管病患者中，缺乏对补体的抑制作用，允许补体激活。
Several complement proteins interact with hemostatic factors. We discovered that von Willebrand factor (VWF) acts as a cofactor for factor I-mediated cleavage of complement C3b, thereby shutting down complement activation. The complement regulatory function of VWF multimers depends on their size. Smaller VWF multimers enhance cleavage of C3b but large and ultra-large VWF (ULVWF) multimers have no effect on C3b cleavage and permit default complement activation. We conclude that normal plasma VWF multimers prevent complement activation and steer the complement pathway toward generation of inactivated C3b (iC3b). ULVWF multimers, as are present in patients with thrombotic microangiopathy, lack an inhibitory effect on complement and permit complement activation.