Definition

A recombinant human form of a disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13) that may be used as a replacement therapy for ADAMTS13 with indirect anti-thrombotic and anti-thrombocytopenic activities. Upon administration, apadamtase alfa acts similarly as endogenous ADAMTS13 and mediates the cleavage of von Willebrand factor (VWF) at the Tyr1605-Met1606 bond in the A2 domain. The cleavage of ultralarge (UL) VWF multimers into smaller forms helps regulate the size of VWF multimers, preventing the binding of platelets to UL VWF and the resulting thrombotic thrombocytopenic purpura (TTP). ADAMTS13 plays a key role in the proteolysis of VWF multimers. VWF is involved in platelet adhesion and blood coagulation.