ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13)—also known as von Willebrand factor-cleaving protease (VWFCP)—is a zinc-containing metalloprotease enzyme that cleaves von Willebrand factor (vWf), a …

Aug 30, 2018 · ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, 13) is a metalloprotease responsible for cleavage of ultra-large von Willebrand factor (VWF) multimers. Severely deficient activity of the protease can trigger an acute episode of thrombotic thrombocytopenic purpura (TTP).

Dec 1, 2016 · The activity of VWF is modulated by ADAMTS13 (a disintegrin and metalloprotease with thrombospondin motif repeats 13), a metalloprotease that cleaves highly procoagulant, ultralarge VWF multimers into smaller, less procoagulant forms.

ADAMTS13 is primarily synthesized in the liver, and its main function is to cleave von Willebrand factor (VWF) anchored on the endothelial surface, in circulation, and at the sites of vascular injury.

They have shown that unfolded, activated ADAMTS‐13 (GoF ADAMTS‐13, the truncated variant MDTCS and WT ADAMTS‐13 activated by VWF D4‐CK) can directly cleave the Aα chain of fibrinogen, rendering fibrin formed in thrombi more prone to lysis by the fibrinolytic system.

ADAMTS13 is a zinc-containing metalloprotease that cleaves von Willebrand factor (VWF). Deficiency of plasma ADAMTS13 activity is accountable for a potentially fatal blood disorder thrombotic thrombocytopenic purpura (TTP). Understanding of ADAMTS13–VWF interaction is essential for developing novel treatments to this disorder.

Recently, it has been shown that ADAMTS-13 undergoes a conformational change upon interaction with VWF, and that this enhances its activity towards its substrate. This review summarizes recent work on these conformational transitions, describing how they are controlled.

Apr 23, 2023 · Low ADAMTS-13 and high VWF have been associated with cardiovascular disease and atrial fibrillation (AF). We aimed to investigate whether VWF, ADAMTS-13, and TSP-1 are associated with clinical outcome. Elderly patients with a recent myocardial infarction (MI) (n = 1027) were followed for 2 years.

Mar 14, 2025 · The authors report that VWF deficiency reduced the severity of ischemic stroke, whereas ADAMTS13 deficiency exacerbated it, demonstrating the role of hyperadhesive VWF in the pathogenesis of ischemic stroke.

Oct 1, 2016 · ADAMTS13 is primarily synthesized by hepatic stellate cells (HSCs), and mainly regulates thrombogenesis by cleaving VWF. Recent studies demonstrate that ADAMTS13 also plays a role in the down-regulation of inflammation, regulation angiogenesis, and degradation of extracellular matrix.