Von Willebrand factor cleaving protease

Desmopressin induces factor VIII and von Willebrand factor (vWF) activity, resulting in an increase in ultra-large vWF multimers. In thrombotic thrombocytopenic purpura there is a deficiency in the von Willebrand factor-cleaving protease (ADAMTS13), which is responsible for degradation of vWF multimers. These ultra-large vWF multimers accumulate and facilitate platelet adhesion. 

Tsai HM Lian EC (1998) Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med, 339 1585-1594. 

Furlan M, Robles R, Galbusera M et al (1998) Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 339 1578-1584... 

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