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首页> 外文期刊>British Journal of Haematology >Indications for a protective function of beta2-glycoprotein I in thrombotic thrombocytopenic purpura
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Indications for a protective function of beta2-glycoprotein I in thrombotic thrombocytopenic purpura

机译：指示β2-糖蛋白I在血栓性血小板减少性紫癜中的保护功能

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摘要

It has been shown that β 2-glycoprotein I (β 2GPI) interacts with von Willebrand factor (VWF) in a glycoprotein (GP)Ib binding state. Given the presence of active VWF multimers in thrombotic thrombocytopenic purpura (TTP), we speculated that β 2GPI might play a role in TTP. We found that β 2GPI plasma levels were significantly lower in acute and remission TTP patients than in normal controls, showing a direct correlation with ADAMTS 13 levels and an inverse correlation with the extent of VWF activation. In vitro flow experiments demonstrated that β 2GPI can block platelet adhesion to endothelial cell-derived VWF strings. We confirmed the direct binding of β 2GPI to VWF by surface plasmon resonance, and determined that domain I of β 2GPI is the binding site of VWF A1 domain. Adhesion of β 2GPI to erythrocytes and platelets was increased in the presence of active VWF, indicating that β 2GPI may be cleared from the circulation during TTP episodes together with blood cells. Our findings suggest that β 2GPI may protect from the effects of hyper-functional VWF by inhibiting its interaction with platelets.
机译：已经表明，β2-糖蛋白I（β2GPI）在糖蛋白（GP）Ib结合状态下与von Willebrand因子（VWF）相互作用。鉴于血栓性血小板减少性紫癜（TTP）中存在活性VWF多聚体，我们推测β2GPI可能在TTP中起作用。我们发现，急性和缓解期TTP患者的β2GPI血浆水平显着低于正常对照组，显示与ADAMTS 13水平直接相关，而与VWF激活程度呈负相关。体外流动实验表明，β2GPI可以阻止血小板粘附于内皮细胞衍生的VWF弦。我们通过表面等离子体共振证实了β2GPI与VWF的直接结合，并确定β2GPI的结构域I是VWF A1域的结合位点。在存在活性VWF的情况下，β2GPI对红细胞和血小板的粘附性增加，表明β2GPI可能在TTP发作期间与血液细胞一起从循环中清除。我们的发现表明，β2GPI可能通过抑制其与血小板的相互作用而免受超功能性VWF的影响。

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