- ? Cell - Endothelial Cell - Megakaryocyte
نویسنده
چکیده
Normal human plasma contains a complex of two proteins that are important in hemostasis and coagulation. The factor VIII procoagulant protein (antihemophilic factor) and the factor VIll-related protein (von Willebrand factor) are under separate genetic control, have distinct biochemical and immunologic properties, and have unique and essential T HE IMPORTANCE of factor VIII in hemostasis and blood coagulation is obvious from the clinical problems in the factor VIII deficiency diseases, classic hemophilia, and von Willebrand’s disease. During the past decade there has been intense research interest in these diseases, the two most common hereditary bleeding disorders, and in the properties of factor VIII. These studies have led to an evolving understanding of factor VIII structure and function. The concept that factor VIII has two distinct biologic functions-coagulant activity and a role in primary hemostasis-was first suggested as an explanation for the dual defect in von Willebrand’s disease.’ The logical inference that factor VIII is a bifunctional molecule was strengthened by reports that proteins purified from human and bovine plasmas had both factor VIII procoagulant activity and the capacity to interact with platelets in a way that might reflect an in vivo role in primary hemostasis.25 Subsequent studies have suggested an alternative interpretation, and it is now generally accepted that plasma factor VIII is a complex of two components that have distinct functions, biochemical and immunologic properties, and genetic control. The properties of these components are summarized in Table I and the relationship is illustrated in Fig. I. One component of the factor VIII complex has antihemophilic factor procoagulant activity and is now usually designated VlII:C. It is inactivated by human antibodies and can be measured (as VllI:CAg) when these reagents are used for immunoassays. The other, physiologic functions. While the nature of their interaction and the details of the biochemical structures remain to be determined, the information now available permits a preliminary understanding of the molecular defects in hemophilia and von Willebrand’s disease.
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