Intravascular blood clots form in an environment in which hydrodynamic forces dominate
and in which fluid-mediated transport is the primary means of moving material. The clotting …

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 …

Normal hemostasis requires von Willebrand factor (VWF) to support platelet adhesion and
aggregation at sites of vascular injury. VWF is a multimeric glycoprotein built from identical …

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

Abstract von Willebrand factor (VWF) is amongst others synthesized by endothelial cells and
stored as ultra-large (UL) VWF multimers in Weibel-Palade bodies. Although UL-VWF is …

Background Type G immunoglobulins against ADAMTS13 are the primary cause of acquired
(idiopathic) thrombotic thrombocytopenic purpura. However, the domains of ADAMTS13 …

Summary Weibel–Palade bodies (WPBs) are the storage organelles for von Willebrand
factor (VWF) in endothelial cells. VWF forms multimers that assemble into tubular structures …

Previous studies have shown that ADAMTS13 spacer domain is required for cleavage of von
Willebrand factor (VWF). However, the exact amino acid residues within this domain critical …

Previous studies have demonstrated that factor VIII (FVIII) or platelets alone increase
cleavage of von Willebrand factor (VWF) by ADAMTS13 under mechanically induced shear …

Platelet-decorated von Willebrand factor (VWF) strings anchored to the endothelial surface
are rapidly cleaved by ADAMTS13. Individual VWF string characteristics such as number …