(en) ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 repeats, member 13) is responsible for the proteolysis of ultra-large von Willebrand factor (VWF) multimers into smaller multimers.[1] Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) caused by severe ADAMTS13 deficiency (activity < 10%) leading to the formation of platelet-rich microthrombi within small arterioles responsible for the destruction of platelets and erythrocytes. [...]
Favresse, J., Lardinois, B., Chatelain, B., Jacqmin, H., & Mullier, F. (2018). Evaluation of the Fully Automated HemosIL Acustar ADAMTS13 Activity Assay. Thrombosis and Haemostasis : international journal for vascular biology and medicine, 118(5), 942-944. https://doi.org/10.1055/s-0038-1641151 (Original work published 2018)