Physiological Roles of the von Willebrand Factor-Factor VIII Interaction

Von Willebrand factorVon Willebrand Factor (VWF) (VWF) and coagulationCoagulation factorCoagulation factor VIII (FVIII) VIII (FVIII) circulate as a complex in plasma and have a major role in the hemostatic system. VWFVon Willebrand Factor (VWF) has a dual role in hemostasisHemostasis. It promotes plateletPlatelet adhesion by anchoring the plateletsPlatelet to the subendothelial matrix of damaged vessels and it protects FVIII from proteolytic degradation. Moreover, VWFVon Willebrand Factor (VWF) is an acute phaseProteins proteinAcute phase protein that has multiple roles in vascular inflammationInflammation and is massively secreted from Weibel-Palade bodies upon endothelial cell activation. Activated FVIII on the other hand, together with coagulationCoagulation factor IX forms the tenase complex, an essential feature of the propagation phase of coagulationCoagulation on the surface of activated plateletsPlatelet. VWFVon Willebrand Factor (VWF) deficiency, either quantitative or qualitative, results in von Willebrand disease (VWD), the most common bleeding disorder. The deficiency of FVIII is responsible for HemophiliaHemophilia A, an X-linked bleeding disorder. Here, we provide an overview on the role of theVon Willebrand Factor (VWF) VWF-FVIII interaction in vascular physiology.