Aug 30, 2018 · ADAMTS13 deficiency is the cause of TTP but also has a significant role in the pathophysiology, diagnosis, and prognosis of other TMAs and thrombotic disorders, such as stroke and myocardial infarction.

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 …

The ADAMTS13 gene provides instructions for making an enzyme that is involved in regulating blood clotting. After an injury, clots normally protect the body by sealing off damaged blood vessels and preventing further blood loss.

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.

Dec 8, 2012 · In the majority of TTP patients, ADAMTS13, the principal regulator of VWF size, is severely deficient. Today, 2 forms of severe ADAMTS13 deficiency are recognized. The acquired form is caused by circulating autoantibodies inhibiting ADAMTS13 activity or increasing ADAMTS13 clearance.

In Panel A, in normal subjects, ADAMTS-13 (von Willebrand factor–cleaving metalloprotease) molecules attach to binding sites on endothelial cell surfaces and cleave unusually large multimers of von Willebrand factor as they are secreted by stimulated endothelial cells.

ADAMTS13, a reprolysin-like metalloprotease, limits platelet-rich thrombus formation in the small arteries by cleaving von Willebrand factor (vWF) at the Tyr1605-Met1606 peptide bond.

ADAMTS13 is an enzyme that acts by cleaving prothrombotic von Willebrand factor (VWF) multimers from the vasculature in a highly regulated manner. In pathologic states such as thrombotic thrombocytopenic purpura (TTP) and other thrombotic microangiopathies (TMAs), VWF can bind to the endothelium and …

Mar 30, 2025 · ADAMTS13 (ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13) is a Protein Coding gene. Diseases associated with ADAMTS13 include Thrombotic Thrombocytopenic Purpura, Hereditary and Thrombotic Thrombocytopenic Purpura.

Aug 1, 2016 · Measurements of ADAMTS13 activity, ADAMTS13 inhibitor, and ADAMTS13 autoantibody are useful for diagnosing TTP, guiding therapy, and predicting relapse. Thrombotic thrombocytopenic purpura (TTP) is an uncommon, life-threatening disease requiring prompt diagnosis and initiation of therapeutic plasma exchange to improve patient survival.