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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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Total citations by year

Year: 2025 2024 2023 2022 2021 2020 2019 2018 2017 2016 2015 2014 2013 2012 2011 2010 2009 2008 2007 2006 Total
Citations: 2 3 5 5 4 3 3 5 5 6 5 4 8 16 12 7 13 8 3 8 125
Citation information
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Citations to this article in year 2009 (13)

Title and authors Publication Year
Pulmonary surfactant: an immunological perspective
ZC Chroneos, Z Sever-Chroneos, VL Shepherd
Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology 2009
Platelet–bacterial interactions
SW Kerrigan, D Cox
Cellular and Molecular Life Sciences 2009
Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site
TJ Raife, W Cao, BS Atkinson, B Bedell, RR Montgomery, SR Lentz, GF Johnson, XL Zheng
Blood 2009
von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental stroke
BQ Zhao, AK Chauhan, M Canault, IS Patten, JJ Yang, M Dockal, F Scheiflinger, DD Wagner
Blood 2009
Shiga toxin-2 results in renal tubular injury but not thrombotic microangiopathy in heterozygous factor H-deficient mice
D Paixão-Cavalcante, M Botto, HT Cook, MC Pickering
Clinical & Experimental Immunology 2009
An autoantibody epitope comprising residues R660, Y661, and Y665 in the ADAMTS13 spacer domain identifies a binding site for the A2 domain of VWF
W Pos, JT Crawley, R Fijnheer, J Voorberg, DA Lane, BM Luken
Blood 2009
Elevated procalcitonin and C-reactive protein as potential biomarkers of sepsis in a subpopulation of thrombotic microangiopathy patients
YO Erickson, NI Samia, B Bedell, KD Friedman, BS Atkinson, TJ Raife
Journal of Clinical Apheresis 2009
Inherited thrombotic thrombocytopenic purpura
M Galbusera, M Noris, G Remuzzi
Haematologica 2009
Mechanisms of microvascular thrombosis in thrombotic thrombocytopenic purpura
HM Tsai
Kidney international. Supplement 2009
Correction of murine ADAMTS13 deficiency by hematopoietic progenitor cell-mediated gene therapy
P Laje, D Shang, W Cao, M Niiya, M Endo, A Radu, N DeRogatis, F Scheiflinger, PW Zoltick, AW Flake, XL Zheng
Blood 2009
Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989–2008)
A Zakarija, HC Kwaan, JL Moake, N Bandarenko, DK Pandey, JM McKoy, PR Yarnold, DW Raisch, JL Winters, TJ Raife, JF Cursio, TH Luu, EA Richey, MJ Fisher, TL Ortel, MS Tallman, XL Zheng, M Matsumoto, Y Fujimura, CL Bennett
Kidney international. Supplement 2009
Molecular basis of ADAMTS13 dysfunction in thrombotic thrombocytopenic purpura
M Manea, D Karpman
Pediatric Nephrology 2009
Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children
C Loirat, JP Girma, C Desconclois, P Coppo, A Veyradier
Pediatric Nephrology 2009

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