Hemophilic dog model for evaluating therapeutic effectiveness of plasma protein fractions.

The Pathogenesis of Hemophilia

It has been possible to duplicate in the hemophilic dog four of the major experiments which have suggested in humans an "anticephalin" hypothesis for the pathogenesis of hemophilia. The experiments in the dog have been considerably extended, as compared with the human experiments, by a variety of techniques. I. Asbestos was placed in contact with hemophilic dog plasma, and the clotting time bec...

Purified human factor VIII procoagulant protein: comparative hemostatic response after infusions into hemophilic and von Willebrand disease dogs.

The procoagulant protein F.VIII:C is noncovalently bound to von Willebrand factor (vWF) to give the factor VIII macromolecular complex. New highly purified preparations of isolated human F.VIII:C, devoid of vWF and about 500,000-fold purified, were administered to hemophilia A and von Willebrand disease (vWD) dogs to determine their hemostatic effectiveness and survival in the circulation. Two ...

Studies of hemophilia; the assay of the antihemophilic clot-promoting principle in normal human plasma with some observations on the relative potency of certain plasma fractions.

Ever since Sahli ( 1 ) demonstrated that the coagulation of hemophilic blood is accelerated by the addition of normal blood 2 many workers (2 to 9) have focused attention on its clot-promoting property. Since cell-free plasma seemed as effective as platelet-rich plasma this action could not be directly attributed to any of the cellular components of blood (2). Hemophilic plasma ws found to be i...

The defect in hemophilic and von Willebrand's disease plasmas studied by a recombination technique.

Factor VIII in preparations from normal plasma is a large glycoprotein of greater than 2 million molecular weight which elutes in the exclusion volume of 4% agarose gels at an ionic strength of 0.15. Recent studies have demonstrated that the factor VIII in canine and bovine plasma is a macromolecular complex composed of a large inert carrier protein and a noncovalently bound small fragment whic...

Chemical, Clinical and Immunological Studies on the Products of Human Plasma Fractionation: Xxxii. the Coagulation Defect in Hemophilia. an in Vitro and in Vivo Comparison of Normal and Hemophilic Whole Blood, Plasma and Derived Plasma Protein Fractions.