Effects of anti-β2GPI antibodies on VWF release from human umbilical vein endothelial cells and ADAMTS13 activity

Christopher J. Ng, Keith R. McCrae, Katrina Ashworth, Lucas J. Sosa, Venkaiah Betapudi, Marilyn J. Manco-Johnson, Alice Liu, Jing Fei Dong, Dominic Chung, Tara C. White-Adams, José A. López, Jorge Di Paola

Research output: Contribution to journalArticlepeer-review

8 Scopus citations


Essentials The antiphospholipid syndrome predisposes to thrombosis due to activation of endothelium and blood components. The role of anti-β2GPI antibodies in VWF release and ADAMTS13 function is not well understood. Some anti-β2GPI antibodies induce endothelial release of soluble VWF but not VWF strings. An anti-β2GPI antibody can decrease ADAMTS13 activity in vitro similar to ex vivo results. Background: Antiphospholipid syndrome (APS) is characterized by recurrent thromboembolic events in the setting of pathologic autoantibodies, some of which are directed to β2-Glycoprotein 1 (β2GPI). The mechanisms of thrombosis in APS appear to be multifactorial and likely include a component of endothelial activation. Among other things, activated endothelium secretes von Willebrand factor, a hemostatic protein that in excess can increase the risk of thrombosis. Objective: We hypothesized that anti-β2GPI antibodies could regulate the release and modulation of VWF from endothelial cells. Patients/Methods: Isolated anti-β2GPI antibodies from patients with APS were assayed for their ability to induced VWF release from HUVECs and modulate the effects of ADAMTS13 in a shear-dependent assay. Results: We observed that anti-β2GPI antibodies from some patients with APS induced VWF release from human endothelial cells but did not induce formation of cell-anchored VWF-platelet strings. Finally, we also determined that one of the Anti-β2GPI antibodies tested can inhibit the function of ADAMTS13, the main modulator of extracellular VWF. Conclusions: These results suggest that VWF and ADAMTS13 may play a role in the prothrombotic phenotype of APS.

Original languageEnglish
Pages (from-to)380-389
Number of pages10
JournalResearch and Practice in Thrombosis and Haemostasis
Issue number2
StatePublished - 2018


  • ADAMTS13
  • antiphospholipid syndrome
  • endothelial cells
  • von Willebrand factor


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