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- W2076618516 abstract "Thrombotic thrombocytopenic purpura (TTP) is characterized by thrombocytopenia and microangiopathic hemolysis. Unlike the typical hemolytic uremic syndrome (HUS), which follows infection with shiga toxin-producing microorganisms, most cases of TTP do not have an obvious etiology. Recent studies revealed that a plasma zinc metalloprotease ADAMTS (a disintegrin and metalloprotease with thrombospondin type 1 motif) 13 cleaves von Willebrand factor in a shear-dependent manner. Deficiency of ADAMTS13, due to autoimmune inhibitors of the protease or genetic mutation in the ADAMTS13 gene, results in a propensity to the development of von Willebrand factor-platelet aggregation and intravascular thrombosis characteristic of TTP. The identification of the molecular defect in TTP raises the prospect that this hitherto mysterious disorder will be managed with a more rationally designed strategy in the near future." @default.
- W2076618516 created "2016-06-24" @default.
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- W2076618516 date "2004-10-01" @default.
- W2076618516 modified "2023-10-18" @default.
- W2076618516 title "Molecular Mechanisms in Thrombotic Thrombocytopenic Purpura" @default.
- W2076618516 doi "https://doi.org/10.1055/s-2004-835675" @default.
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