ReviewJournal of thrombosis and haemostasis : JTH2022
Mechanisms of ADAMTS13 regulation.
Review in Journal of thrombosis and haemostasis : JTH, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers.
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The trial behind it
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Who cites it
36 citing papers in PubMed, 56 citations in OpenAlex.
- Hematologic complications and design challenges in cardiovascular devices.Communications engineering · 2026Review
- A rapid ADAMTS13 activity assay using a portable readout device.Blood vessels, thrombosis & hemostasis · 2026Article
- Prognostic value of von Willebrand factor and ADAMTS-13 in patients with sepsis-induced coagulopathy.Research and practice in thrombosis and haemostasis · 2026Article
- Dried plasma retains hemostatic function and thermal stability during Arctic military operations.Transfusion · 2026Article
- On a quest for fingerprints of crystalglobulinemia on platelets and endothelium.Blood vessels, thrombosis & hemostasis · 2026Article
- Novel cryptic ADAMTS13 epitopes uncover a distinct open ADAMTS13 conformation in immune-mediated TTP.Haematologica · 2026Article
- Case Series and Literature Narrative Review of Immune-Mediated Thrombotic Thrombocytopenic Purpura in Children.Children (Basel, Switzerland) · 2026Article
- Von Willebrand Factor and ADAMTS13 in Relation to Major Adverse Limb Events in Peripheral Artery Disease.Vascular specialist international · 2026Article
- The ADAMTS family: from extracellular matrix proteases to orchestrators of fibrosis.Cell communication and signaling : CCS · 2026Review
- Optical-Tweezer Single-Molecule Assay for ADAMTS13-Mediated Proteolysis of the von Willebrand Factor A2 Domain.Methods in molecular biology (Clifton, N.J.) · 2026Article
- Endotheliopathy in CAR T-Cell Therapy: Mechanistic Insights into the VWF/ADAMTS13 Axis and the Angiopoietin-Tie2 Pathway.TH open : companion journal to thrombosis and haemostasis · 2026Review
- Systematic Literature Review of the Efficacy and Safety of Treatments in Congenital Thrombotic Thrombocytopenic Purpura.Journal of blood medicine · 2026Review
- Deep Protease Profiling to Define the Substrate Specificity of ADAMTS Proteases.Methods in molecular biology (Clifton, N.J.) · 2026Article
- ADAMTS13 in Bothrops lanceolatus snakebite envenoming: Crude venom-induced reduction of in vitro enzymatic activity and clinical correlation in snakebite patients.PLoS neglected tropical diseases · 2025Article
- Can Plasma ADAMTS13 Differentiate Patients With Pulmonary Arterial Hypertension From Other Forms of Pulmonary Hypertension and Dyspnea Control Patients.CHEST pulmonary · 2025Article
- Open or closed? Understanding the molecular mechanisms and clinical implications of ADAMTS13's conformation.HemaSphere · 2025Review
- Development of a protease-resistant ADAMTS13 to improve stability against proteolytic degradation.Blood advances · 2025Article
- Freeze-dried plasma: Hemostasis and biophysical analyses for damage control resuscitation.Transfusion · 2025Article
- A Disintegrin and Metalloprotease with Thrombospondin Motif, Member 13, and Von Willebrand Factor in Relation to the Duality of Preeclampsia and HIV Infection.International journal of molecular sciences · 2025Review
- Agents of shield: glycans mask antibody epitopes on ADAMTS13.Blood advances · 2025Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
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 recombinant ADAMTS13 may have broad applicability as an antithrombotic agent. Despite this progress, we currently do not understand the mechanisms that regulate ADAMTS13 activity in vivo. ADAMTS13 evades canonical means of protease regulation because it is secreted as an active enzyme and has a long half-life in circulation, suggesting that it is not inhibited by natural protease inhibitors. Although shear can spatially and temporally activate von Willebrand factor to capture circulating platelets, it is also required for cleavage by ADAMTS13. Therefore, spatial and temporal regulation of ADAMTS13 activity may be required to stabilize von Willebrand factor-platelet strings at sites of vascular injury. This review outlines potential mechanisms that regulate ADAMTS13 in vivo including shear-dependency, local inactivation, and biochemical and structural regulation of substrate binding. Recently published structural data of ADAMTS13 is discussed, which may help to generate novel hypotheses for future research.
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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.