Platelet activation and the formation of the platelet plug: deficiency of ADAMTS13 causes thrombotic thrombocytopenic purpura.
In the circulation, a plasma metalloprotease, ADAMTS13, 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.
Tsai
Han-Mou
HM
Division of Hematology, Montefiore Medical Center and Albert Einstein College of Medicine, 111 E 210th St, Bronx, NY, USA. htsai@montefiore.org
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D002648:Child; D004721:Endoplasmic Reticulum / Q000378:metabolism; D006463:Hemolytic-Uremic Syndrome / Q000503:physiopathology; D006801:Humans; D008666:Metalloendopeptidases / Q000172:deficiency* / Q000235:genetics; D009154:Mutation; D015539:Platelet Activation; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism*; D013314:Stress, Mechanical; D013927:Thrombosis / Q000378:metabolism; D014841:von Willebrand Factor / Q000378:metabolism
D014841:von Willebrand Factor; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human