ArticleBlood2009
The distal carboxyl-terminal domains of ADAMTS13 are required for regulation of in vivo thrombus formation.
Article in Blood, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers.
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Who cites it
39 citing papers in PubMed, 77 citations in OpenAlex.
- Residues Arg568 and Phe592 contribute to an antigenic surface for anti-ADAMTS13 antibodies in the spacer domain.Haematologica · 2011Trial
- Pathological Allostery in ADAMTS13: Autoantibody-Induced Modulation and Its Role in Immune Thrombotic Thrombocytopenic Purpura (iTTP).Pharmaceuticals (Basel, Switzerland) · 2026Review
- Development of a protease-resistant ADAMTS13 to improve stability against proteolytic degradation.Blood advances · 2025Article
- Optimization of plasma-based BioID identifies plasminogen as a ligand of ADAMTS13.Scientific reports · 2024Article
- Animal models for thrombotic thrombocytopenic purpura: a narrative review.Annals of blood · 2023Article
- Immune thrombotic thrombocytopenic purpura: pathogenesis and novel therapies: a narrative review.Annals of blood · 2023Article
- Mechanisms of ADAMTS13 regulation.Journal of thrombosis and haemostasis : JTH · 2022Review
- Residues R1075, D1090, R1095, and C1130 Are Critical in ADAMTS13 TSP8-Spacer Interaction Predicted by Molecular Dynamics Simulation.Molecules (Basel, Switzerland) · 2021Article
- Exome Sequencing Identifies Abnormalities in Glycosylation and ANKRD36C in Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura.Thrombosis and haemostasis · 2021Article
- Domain-specific mechanical modulation of VWF-ADAMTS13 interaction.Molecular biology of the cell · 2019Article
- ADAMTS13 Deficiency Worsens Colitis and Exogenous ADAMTS13 Administration Decreases Colitis Severity in Mice.TH open : companion journal to thrombosis and haemostasis · 2017Article
- Removal of the C-Terminal Domains of ADAMTS13 by Activated Coagulation Factor XI induces Platelet Adhesion on Endothelial Cells under Flow Conditions.Frontiers in medicine · 2017Article
- ADAMTS13 autoantibodies cloned from patients with acquired thrombotic thrombocytopenic purpura: 1. Structural and functional characterization in vitro.Transfusion · 2016Article
- ADAMTS13 and von Willebrand factor interactions.Current opinion in hematology · 2015Article
- Nxf1 natural variant E610G is a semi-dominant suppressor of IAP-induced RNA processing defects.PLoS genetics · 2015Article
- ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.Annual review of medicine · 2015Review
- Allosteric activation of ADAMTS13 by von Willebrand factor.Proceedings of the National Academy of Sciences of the United States of America · 2014Article
- Article
- Von Willebrand Factor Abnormalities Studied in the Mouse Model: What We Learned about VWF Functions.Mediterranean journal of hematology and infectious diseases · 2013Article
- Protective anti-inflammatory effect of ADAMTS13 on myocardial ischemia/reperfusion injury in mice.Blood · 2012Article
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Authors and funding
7 authors at 2 institutions in 2 countries.
Funding
Abstract
ADAMTS13 is a multidomain protease that limits platelet thrombogenesis through the cleavage of von Willebrand factor (VWF). We previously identified 2 types of mouse Adamts13 gene: the 129/Sv-strain Adamts13 gene encodes the long-form ADAMTS13 having the same domains as human ADAMTS13, whereas the C57BL/6-strain Adamts13 gene encodes the short-form ADAMTS13 lacking the distal C-terminal domains. To assess the physiologic significance of the distal C-terminal domains of ADAMTS13, we generated and analyzed 129/Sv-genetic background congenic mice (Adamts13(S/S)) that carry the short-form ADAMTS13. Similar to wild-type 129/Sv mice (Adamts13(L/L)), Adamts13(S/S) did not have ultralarge VWF multimers in plasma, in contrast to 129/Sv-genetic background ADAMTS13-deficient mice (Adamts13(-/-)). However, in vitro thrombogenesis under flow at a shear rate of 5000 s(-1) was accelerated in Adamts13(S/S) compared with Adamts13(L/L). Both in vivo thrombus formation in ferric chloride-injured arterioles and thrombocytopenia induced by collagen plus epinephrine challenge were more dramatic in Adamts13(S/S) than in Adamts13(L/L) but less than in Adamts13(-/-). These results suggested that the C-terminally truncated ADAMTS13 exhibited decreased activity in the cleavage of VWF under high shear rate. Role of the C-terminal domains may become increasingly important under prothrombotic conditions.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.