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Mechanisms of ADAMTS13 regulation

Journal of Thrombosis and Haemostasis, 2022
Recombinant ADAMTS13 is currently undergoing clinical trials as a treatment for hereditary thrombotic thrombocytopenic purpura, a lethal microvascular condition resulting from ADAMTS13 deficiency. Preclinical studies have also demonstrated its efficacy in treating arterial thrombosis and inflammation without causing bleeding, suggesting that ...
Veronica DeYoung, Kanwal Singh, Colin A. Kretz   +2 more
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ADAMTS13 and Non-ADAMTS13 Biomarkers in Immune-Mediated Thrombotic Thrombocytopenic Purpura

Journal of Clinical Medicine, 2023
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare medical emergency for which a correct and early diagnosis is essential. As a severe deficiency in A Disintegrin And Metalloproteinase with ThromboSpondin type 1 repeats, member 13 (ADAMTS13) is the underlying pathophysiology, diagnostic strategies require timely monitoring of ADAMTS13
Quintijn Bonnez, Kazuya Sakai, Karen Vanhoorelbeke   +2 more
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ADAMTS13 activity and inhibitor [PDF]

American Journal of Hematology, 2008
AbstractThrombotic thrombocytopenic purpura (TTP) is often associated with acquired or congenital deficiency of the von Willebrand factor‐cleaving metalloprotease, ADMATS13 (Lammle B et al., J Thromb Haemost 2005;3:1663‐1675; Schneppenheim et al., Blood 2003;101:1845‐1850).
Jonathan K. Freeman, Vandita Johari, Adriana Doldan-Silvero, Clandine Habib, Carlos Acevedo-Gadea   +4 more
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The VWF/ADAMTS13 axis in the antiphospholipid syndrome: ADAMTS13 antibodies and ADAMTS13 dysfunction [PDF]

British Journal of Haematology, 2008
SummaryAutoantibodies to ADAMTS13 (a disintegrin‐like and metalloprotease with thrombospondin type I motif, member 13) play an important role in the development of microthrombosis in thrombotic thrombocytopenic purpura (TTP). In severe cases of antiphospholipid syndrome (APS), microthrombosis can occur similar to that seen in TTP, suggesting possible ...
Richard D. Starke, Samuel J. Machin, Hannah Cohen, Anthony Lawrie, I. J. Mackie, S. K. Austin   +5 more
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Conformational activation of ADAMTS13 [PDF]

Proceedings of the National Academy of Sciences, 2014
Significance We show that a CUB–spacer domain interaction impedes exposure of the ADAMTS13 spacer functional exosite, preventing ADAMTS13 from interacting effectively with its complementary binding site in the VWF A2 domain. This CUB–spacer interaction is disrupted by interaction with the C-terminal domains of VWF, leading to conformational ...
South, K, Luken, BM, Crawley, JTB, Phillips, R, Thomas, M, Collins, RF, Deforche, L, Vanhoorelbeke, K, Lane, DA   +8 more
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Mechanistic Studies on ADAMTS13 Catalysis [PDF]

Biophysical Journal, 2008
The zinc-protease a disintegrin-like and metalloprotease with thrombospondin type I repeats (ADAMTS13) cleaves the Tyr(1605)-Met(1606) peptide bond of von Willebrand factor (VWF), avoiding the accumulation of ultra large VWF multimers. Hydrolysis by ADAMTS13 of a VWF analog (Asp(1596)-Arg(1668) peptide, fluorescence energy transfer substrate [FRETS ...
Pier Mannuccio Mannucci, Roberta Palla, Enrico Di Stasio, Raimondo De Cristofaro, Raimondo De Cristofaro, Flora Peyvandi, Stefano Lancellotti   +6 more
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Disappearing acts of ADAMTS13

EBioMedicine, 2015
The study by Thomas and co-workers in this issue of EBioMedicine provides detailed characteristics on the pathogenic properties of antibodies that develop in patients with acquired thrombotic thrombocytopenic purpura (TTP) (Thomas et al., 2015). It is now well-established that TTP is due to dysregulation of primary hemostasis. A crucial initial step in
Rob Fijnheer, Jan Voorberg, Fabian C. Verbij   +2 more
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ADAMTS13 and microvascular thrombosis [PDF]

Expert Review of Cardiovascular Therapy, 2006
Interaction between platelet and von Willebrand factor, a circulating adhesive glycoprotein, is essential for hemostasis under the high shear environments of arterioles and capillaries. If unregulated, this interaction may lead to unwarranted platelet thrombosis.
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Genetic variants in the ADAMTS13 and SUPT3H genes are associated with ADAMTS13 activity [PDF]

Blood, 2015
Key Points We identify rs41314453 as the strongest genetic predictor of ADAMTS13 activity, associated with a decrease of >20%. We present evidence of further independent associations with a common variant in SUPT3H, as well as 5 variants at the ADAMTS13 locus.
Moniek P.M. de Maat, André G. Uitterlinden, Michelle A.H. Sonneveld, Johan Boender, Fernando Rivadeneira, Abbas Dehghan, M. Arfan Ikram, Paul S. de Vries, Oscar H. Franco, Hanspeter Rottensteiner, Frank W.G. Leebeek, Albert Hofman   +11 more
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ADAMTS13, TTP and Beyond [PDF]

Hereditary Genetics, 2013
Copyright: © 2012 Zheng XL. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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