Studies of ADAMTS13 expression and activity in the kidney
Von Willebrand factor (VWF) is an abundant plasma glycoprotein involved in platelet adhesion and aggregation at sites of vascular injury. ADAMTS13 is the sole physiological VWF-cleaving protease, thus regulating the size of thrombus growth. Dysfunctional ADAMTS13 leads to thrombotic thrombocytopenic purpura (TTP), which is either due to mutations (congenital TTP) or auto-antibodies (acquired TTP).