Mouse models of thrombotic thrombocytopenic purpura and methods for its use

摘要

A mouse model of a blood clotting disorder, said disorder characterized by the presence of blood clots in blood vessels throughout the body, said mouse model comprising an inability to degrade the von Willebrand factor polypeptide ( Recombinant human VWF) comprising ultra-large multimers, wherein said disorder in said mouse model is caused after intravenous administration of a dose of the recombinant human VWF comprising ultra-large multimers to a mouse, where the dose of the recombinant human VWF is higher. or equal to 500 RCoU / kg of body weight or, if the mouse is deficient in the VWF polypeptide, the dose of the recombinant human VWF is greater than or equal to 1000 RCoU / kg of body weight, where the mouse model shows changes histopathological indicative of disseminated intravascular coagulopathy and / or thrombotic thrombocytopenic purpura, and wherein said mouse model is suitable for testing the efficacy of a therapeutic agent in the treatment of a blood coagulation disorder.