Deficiency of ADAMTS13 causes thrombotic thrombocytopenic purpura. Editorial

In the circulation, a plasma metalloprotease, ADAMTS 13, cleaves von Willebrand factor (vWF) in a shear-dependent manner. This article reviews the role of this cleavage in regulating vWF-platelet interaction and proposes a scheme for understanding how a deficiency of ADAMTS13 results in the development of microthrombi in patients with thrombotic thrombocytopenic purpura.