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    Arterioscler Thromb Vasc Biol. 2003 Mar 1;23(3):388-96. Epub 2003 Jan 30.

    Platelet activation and the formation of the platelet plug: deficiency of ADAMTS13 causes thrombotic thrombocytopenic purpura.

    Tsai HM.

    Division of Hematology, Montefiore Medical Center and Albert Einstein College of Medicine, 111 E 210th St, Bronx, NY, USA. htsai@montefiore.org

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    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.

    PMID: 12615692 [PubMed - indexed for MEDLINE]

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