Platelet factor 4 inhibits ADAMTS13 activity and regulates the multimeric distribution of von Willebrand factor
Platelet factor 4 inhibits ADAMTS13 activity and regulates the multimeric distribution of von Willebrand factor
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DOI：
10.1111/bjh.16553
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发表时间：
2020-03-04
影响因子：
6.5
通讯作者：
Arnold, Donald M.
中科院分区：
文献类型：
--
作者：
Nazy, Ishac;Elliott, Taylor D.;Arnold, Donald M.
血管性血友病因子 (VWF) 在血栓形成中的效率与其多聚体大小有关，而多聚体大小由蛋白酶 ADAMTS13 控制。然而，尚不清楚 ADAMTS13 活性的调节因素。在本研究中，我们研究了 PF4 是否可以与 VWF 结合并抑制 ADAMTS13 活性。我们发现 PF4 与 VWF 结合并防止 ADAMTS13 活性。我们还发现 VWF-PF4 复合物在血栓性血小板减少性紫癜 (TTP) 患者中循环。我们的数据提供了第一个证据，证明 PF4 可能在初级止血和血栓形成过程中调节 VWF 多聚体方面具有新作用。
The efficiency of von Willebrand factor (VWF) in thrombus formation is related to its multimeric size, which is controlled by the protease ADAMTS13. However, it is not clear what regulates ADAMTS13 activity. In this study, we investigated whether PF4 could bind to VWF and inhibit ADAMTS13 activity. We found that PF4 binds to VWF and protects against ADAMTS13 activity. We also found that VWF-PF4 complexes circulate in patients with thrombotic thrombocytopenic purpura (TTP). Our data provides the first evidence that PF4 may have a novel role in regulating VWF multimers during primary haemostasis and thrombosis.