The physiology and pathophysiology of the factor VIII complex

作者: Rob J. Hamer , Wim P.M. Houdijk , Jan J. Sixma , Trevor W. Barrowcliffe

DOI: 10.1016/S1040-8428(86)80046-4

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摘要: The factor VIII complex consists of two noncovalently linked proteins: von Willebrand (VWF) and (FVIII). VWF plays an important role in primary hemostasis by mediating the adherence blood platelets to damaged vessel wall. A review literature on is given with regard its physicochemical properties mode action. FVIII acts as a cofactor Xa-generating enzyme intrinsic coagulation cascade. Starting recently published structure FVIII, reviewed for structural information FVIII. Also, effort made characterize interaction discuss possible physiological significance FVIII-VWF formation. Interaction clotting factors pathway described detail. Hemophilia Willebrand's disease (VWD) are both congenital bleeding disorders affecting great many people. different variants these diseases some reference therapy detection.

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