The physiology and pathophysiology of the factor VIII complex.
The factor VIII complex consists of two noncovalently linked proteins: von Willebrand factor (VWF) and factor VIII (FVIII). VWF plays an important role in primary hemostasis by mediating the adherence of blood platelets to the damaged vessel wall. A review of the literature on VWF is given with regard to its physicochemical properties and mode of action. FVIII acts as a cofactor in the factor Xa-generating enzyme complex of the intrinsic coagulation cascade. Starting with the recently published primary structure of FVIII, the literature is reviewed for structural information on FVIII. Also, an effort is made to characterize the interaction of FVIII with VWF and to discuss the possible physiological significance of FVIII-VWF complex formation. Interaction of FVIII with the clotting factors of the intrinsic pathway of coagulation is described in detail. Hemophilia and von Willebrand's disease (VWD) are both congenital bleeding disorders affecting a great many people. The different variants of these diseases are described with some reference to therapy and detection.
D002241:Carbohydrates / Q000502:physiology; D005169:Factor VIII / Q000502:physiology*; D006467:Hemophilia A / Q000097:blood; D006801:Humans; D010974:Platelet Aggregation; D013329:Structure-Activity Relationship; D014842:von Willebrand Diseases / Q000097:blood; D014841:von Willebrand Factor / Q000032:analysis / Q000502:physiology*
D002241:Carbohydrates; D014841:von Willebrand Factor; D005169:Factor VIII