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Citations to this article

Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice
David G. Motto, … , Denisa D. Wagner, David Ginsburg
David G. Motto, … , Denisa D. Wagner, David Ginsburg
Published October 3, 2005
Citation Information: J Clin Invest. 2005;115(10):2752-2761. https://doi.org/10.1172/JCI26007.
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Research Article Hematology Article has an altmetric score of 3

Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice

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Abstract

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening illness caused by deficiency of the vWF-cleaving protease ADAMTS13. Here we show that ADAMTS13-deficient mice are viable and exhibit normal survival, although vWF-mediated platelet-endothelial interactions are significantly prolonged. Introduction of the genetic background CASA/Rk (a mouse strain with elevated plasma vWF) resulted in the appearance of spontaneous thrombocytopenia in a subset of ADAMTS13-deficient mice and significantly decreased survival. Challenge of these mice with shigatoxin (derived from bacterial pathogens associated with the related human disease hemolytic uremic syndrome) resulted in a striking syndrome closely resembling human TTP. Surprisingly, no correlation was observed between plasma vWF level and severity of TTP, implying the existence of TTP-modifying genes distinct from vWF. These data suggest that microbe-derived toxins (or possibly other sources of endothelial injury), together with additional genetic susceptibility factors, are required to trigger TTP in the setting of ADAMTS13 deficiency.

Authors

David G. Motto, Anil K. Chauhan, Guojing Zhu, Jonathon Homeister, Colin B. Lamb, Karl C. Desch, Weirui Zhang, Han-Mou Tsai, Denisa D. Wagner, David Ginsburg

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Year: 2025 2024 2023 2022 2021 2020 2019 2018 2017 2016 2015 2014 2013 2012 2011 2010 2009 2008 2007 2006 Total
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Citations to this article in year 2023 (5)

Title and authors Publication Year
Is Endothelial Activation a Critical Event in Thrombotic Thrombocytopenic Purpura?
Cauchois R, Muller R, Lagarde M, Dignat-George F, Tellier E, Kaplanski G
Journal of Clinical Medicine 2023
Reversible skin microvascular hyporeactivity in patients with immune-mediated thrombocytopenic thrombotic purpura.
Joffre J, Raia L, Urbina T, Bonny V, Gabarre P, Missri L, Baudel JL, Coppo P, Guidet B, Maury E, Ait-Oufella H
Critical Care 2023
Immune-mediated thrombotic thrombocytopenic purpura plasma induces calcium- and IgG-dependent endothelial activation: correlations with disease severity.
Tellier E, Widemann A, Cauchois R, Faccini J, Lagarde M, Brun M, Robert P, Robert S, Bachelier R, Poullin P, Roose E, Vanhoorelbeke K, Coppo P, Dignat-George F, Kaplanski G
Haematologica 2023
Recombinant ADAMTS-13 Improves Survival of Mice Subjected to Endotoxemia.
Gao D, Zhou Z, Ma R, Wu H, Nguyen T, Liu L, Dong J
International journal of molecular sciences 2023
Coagulation factor VIII regulates von Willebrand factor homeostasis invivo.
Cao W, Trask AR, Bignotti AI, George LA, Doshi BS, Sabatino DE, Yada N, Zheng L, Camire RM, Zheng XL
Journal of Thrombosis and Haemostasis 2023

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