The role of von Willebrand factor in hemostasis pathology
https://doi.org/10.21518/2307-1109-2022-12-2-79-102
Abstract
Von Willebrand factor (VWF) is a multimeric plasma glycoprotein present in endothelial cells, megakaryocytes, platelets, and connective tissue. It mediates platelet adhesion in small arteries. VWF also binds and protects coagulation factor VIII from degradation. Moreover, VWF is involved in inflammatory response, linking hemostasis and inflammation. VWF multimers and platelets attached to damaged or activated endothelium mediate leukocyte recruitment, facilitating local inflammatory response. At shear rates above 5000 s–1, VWF molecules are capable of hydrodynamic activation that changes their conformation from globular to fibrillar. Therefore, VWF plays a key role in cellular hemostasis at high shear rates. Acquired and inherited disfunction, defective synthesis or increased proteolysis of VWF multimers lead to bleeding, as in von Willebrand disease or Heyde syndrome. Pathological activation of VWF may lead to the development of thrombotic complications of coronary artery disease. COVID-19, especially severe form, is characterized by prothrombotic shift in pulmonary vascular bed. Following endothelial damage, VWF plasma level rises and ADAMTS-13 activity decreases. In patients with COVID-19, a change in the VWF/ADAMTS-13 ratio is associated with an increase in the risk of thromboembolic complications. Therefore, assessment of hydrodynamic activation of VWF under flow conditions may be valuable in fundamental research and laboratory diagnostics.
Supporting agencies: The work was supported by the Russian Science Foundation grant No. 21-15-00029.
About the Authors
Yu. N. Avtaeva
National Medical Research Center for Cardiology named after academician E.I. Chazov
Russian Federation
Russian Federation
Yuliya N. Avtaeva, Cand. Sci. (Med.), Junior Researcher, Laboratory of Cellular Hemostasis
15a, 3rd Cherepkovskaya St., Moscow, 121552
I. S. Melnikov
National Medical Research Center for Cardiology named after academician E.I. Chazov; State Scientific Center of the Russian Federation – The Institute of Biomedical Problems of the Russian Academy of Sciences
Russian Federation
Russian Federation
Ivan S. Melnikov, Researcher, Laboratory of Cellular Hemostasis; Junior Researcher, Laboratory of Gas Exchange, Biomechanics and Barophysiology
15a, 3rd Cherepkovskaya St., Moscow, 121552
76a, Khoroshevskoye Shosse, Moscow, 123007
S. A. Vasiliev
National Medical Research Center for Hematology
Russian Federation
Russian Federation
Sergey A. Vasiliev, Dr. Sci. (Med.), Professor, Leading Researcher
44, Novyy Zykovskiy Proezd, Moscow
Z. A. Gabbasov
National Medical Research Center for Cardiology named after academician E.I. Chazov
Russian Federation
Russian Federation
Zufar A. Gabbasov, Dr. Sci. (Biol.), Chief Researcher, Laboratory of Cellular Hemostasis
15a, 3rd Cherepkovskaya St., Moscow, 121552
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For citations:
Avtaeva Yu.N., Melnikov I.S., Vasiliev S.A., Gabbasov Z.A. The role of von Willebrand factor in hemostasis pathology. Aterotromboz = Atherothrombosis. 2022;12(2):79-102. (In Russ.) https://doi.org/10.21518/2307-1109-2022-12-2-79-102
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