ArticleThe Journal of biological chemistry2017
A model for the conformational activation of the structurally quiescent metalloprotease ADAMTS13 by von Willebrand factor.
Article in The Journal of biological chemistry, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 37 citations in OpenAlex.
- Expression of wild-type and recombinant D4-CK domains of VWF gene with novel variants in Pakistan.Molecular biology reports · 2026Article
- The landscape of ADAMTS13-related tests in thrombotic thrombocytopenic purpura: a review.Frontiers in medicine · 2026Review
- Open or closed? Understanding the molecular mechanisms and clinical implications of ADAMTS13's conformation.HemaSphere · 2025Review
- A novel mechanism underlying allosteric regulation of ADAMTS-13 revealed by hydrogen-deuterium exchange plus mass spectrometry.Research and practice in thrombosis and haemostasis · 2023Article
- ADAMTS13 conformations and mechanism of inhibition in immune thrombotic thrombocytopenic purpura.Journal of thrombosis and haemostasis : JTH · 2022Review
- Engineered Molecular Therapeutics Targeting Fibrin and the Coagulation System: a Biophysical Perspective.Biophysical reviews · 2022Review
- Article
- Residues R1075, D1090, R1095, and C1130 Are Critical in ADAMTS13 TSP8-Spacer Interaction Predicted by Molecular Dynamics Simulation.Molecules (Basel, Switzerland) · 2021Article
- Crystal structure of ADAMTS13 CUB domains reveals their role in global latency.Science advances · 2021Article
- Antibodies that conformationally activate ADAMTS13 allosterically enhance metalloprotease domain function.Blood advances · 2020Article
- Insights Into Immunothrombosis: The Interplay Among Neutrophil Extracellular Trap, von Willebrand Factor, and ADAMTS13.Frontiers in immunology · 2020Review
- Identification of cysteine thiol-based linkages in ADAMTS13 in support of a non-proteolytic regulation of von Willebrand factor.Journal of thrombosis and haemostasis : JTH · 2019Article
- Domain-specific mechanical modulation of VWF-ADAMTS13 interaction.Molecular biology of the cell · 2019Article
- Exploring the "minimal" structure of a functional ADAMTS13 by mutagenesis and small-angle X-ray scattering.Blood · 2019Article
- Hairpin and allosteric regulation in ADAMTS13.Blood · 2019Article
- Article
- The ADAMTS13-VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension.The European respiratory journal · 2019Article
- AFM Imaging Reveals Multiple Conformational States of ADAMTS13.Journal of biological engineering · 2019Article
- Enhanced activity of an ADAMTS-13 variant (R568K/F592Y/R660K/Y661F/Y665F) against platelet agglutination in vitro and in a murine model of acute ischemic stroke.Journal of thrombosis and haemostasis : JTH · 2018Article
- Dissecting the pathophysiology of immune thrombotic thrombocytopenic purpura: interplay between genes and environmental triggers.Haematologica · 2018Review
Corrections and comments
- Erratum issued
Authors and funding
3 authors at 2 institutions in 1 country.
Funding
Abstract
Blood loss is prevented by the multidomain glycoprotein von Willebrand factor (VWF), which binds exposed collagen at damaged vessels and captures platelets. VWF is regulated by the metalloprotease ADAMTS13, which in turn is conformationally activated by VWF. To delineate the structural requirements for VWF-mediated conformational activation of ADAMTS13, we performed binding and functional studies with a panel of truncated ADAMTS13 variants. We demonstrate that both the isolated CUB1 and CUB2 domains in ADAMTS13 bind to the spacer domain exosite of a truncated ADAMTS13 variant, MDTCS (
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Registered trials
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.