Pathogenesis of thrombotic thrombocytopenic purpura (TTP) was a mystery for over half a
century until the discovery of ADAMTS13. ADAMTS13 is primarily synthesized in the liver …

Abstract von Willebrand factor (VWF) is a large adhesive glycoprotein with established
functions in hemostasis. It serves as a carrier for factor VIII and acts as a vascular damage …

A disintegrin and metalloproteinase (ADAM) family proteins constitute a major class of
membrane-anchored multidomain proteinases that are responsible for the shedding of cell …

Profound thrombocytopenia and microangiopathic hemolytic anemia characterize
thrombotic microangiopathy, which includes two major disorders: thrombotic …

Congenital thrombotic thrombocytopenic purpura (TTP)(also known as Upshaw‐Schulman
syndrome, USS) is a rare, life‐threatening disease characterized by thrombocytopenia and …

Platelet recruitment to sites of blood vessel damage is highly dependent upon von
Willebrand factor (VWF). VWF platelet-tethering function is proteolytically regulated by the …

Summary ADAMTS‐13, a plasma reprolysin‐like metalloprotease, cleaves von W illebrand
factor (VWF). Severe deficiency of plasma ADAMTS‐13 activity results in thrombotic …

ADAMTS13 specifically cleaves plasma von Willebrand factor (VWF) and thereby controls
VWF-mediated platelet thrombus formation. Severe deficiencies in ADAMTS13 can cause …

Neutrophil NETosis is a unique form of cell death, characterized by the release of
decondensed chromatin and antimicrobial contents to the extracellular space, which is …

Thrombotic thrombocytopenic purpura (TTP) is primarily caused by immunoglobulin G (IgG)
autoantibodies against A Disintegrin And Metalloprotease with ThromboSpondin type 1 …