biostudies-literatureChauhan AKNHLBI NIH HHS767-76https://www.ebi.ac.uk/biostudies/studies/S-EPMC2118248biostudies-literatureUnknown203(3)The metalloprotease ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type I repeats 13) cleaves highly adhesive large von Willebrand factor (VWF) multimers after their release from the endothelium. ADAMTS13 deficiency is linked to a life-threatening disorder, thrombotic thrombocytopenic purpura (TTP), characterized by platelet-rich thrombi in the microvasculature. Here, we show spontaneous thrombus formation in activated microvenules of Adamts13-/- mice by intravital microscopy. Strikingly, we found that ADAMTS13 down-regulates both platelet adhesion to exposed subendothelium and thrombus formation in injured arterioles. An inhibitory antibody to ADAMTS13 infused in wild-type mice prolonged adhesion of platelets to endothelium and induced thrombi formation with embolizaThe Journal of experimental medicineSystemic antithrombotic effects of ADAMTS13.PMC2118248R37 HL041002R37 HL41002R01 HL39693R01 HL041002P01 HL057346R01 HL039693Motto DGPlaimauer BBergmeier WLamb CBScheiflinger FChauhan AKWagner DDGinsburg DDockal MfalseSystemic antithrombotic effects of ADAMTS13.The metalloprotease ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type I repeats 13) cleaves highly adhesive large von Willebrand factor (VWF) multimers after their release from the endothelium. ADAMTS13 deficiency is linked to a life-threatening disorder, thrombotic thrombocytopenic purpura (TTP), characterized by platelet-rich thrombi in the microvasculature. Here, we show spontaneous thrombus formation in activated microvenules of Adamts13-/- mice by intravital microscopy. Strikingly, we found that ADAMTS13 down-regulates both platelet adhesion to exposed subendothelium and thrombus formation in injured arterioles. An inhibitory antibody to ADAMTS13 infused in wild-type mice prolonged adhesion of platelets to endothelium and induced thrombi formation with emboliza2006-01-01T00:00:00Z2006 Mar2026-04-16T05:13:21.045Z2019-03-26T23:29:37ZS-EPMC21182481653388110.1084/jem.20051732