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ADAMTS13 deficiency impairs shear-dependent VWF cleavage, driving platelet microthrombi in thrombotic thrombocytopenic purpura.
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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.
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Han‐Mou Tsai (2003) studied this question. ADAMTS13 deficiency impairs shear-dependent cleavage of von Willebrand factor, leading to dysregulated platelet interaction and microthrombi in thrombotic thrombocytopenic purpura.
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