Transcripts coding the VWF cleaving protease are decreased under proinflammatory conditions, which is reversed by co-incubation with activated protein C and selenate

In sepsis, the severity-dependent decrease of the VWF-cleaving protease ADAMTS13 is a common phenomenon, which may contribute to aggregation of platelets/platelet consumption and the development of sepsis-associated thrombotic microangiopathy (TMA) and organ failure. Up to now, hepatic stellate cells (HSC) are considered to function as the primary source of ADAMTS13 protein. The underlying mechanisms of the decrease in sepsis remain unclear.