ArticleBlood2006
Apical sorting of ADAMTS13 in vascular endothelial cells and Madin-Darby canine kidney cells depends on the CUB domains and their association with lipid rafts.
Article in Blood, 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 57 papers, 2 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
57 citing papers in PubMed, 2 syntheses or guidelines pooled it, 114 citations in OpenAlex.
- Association between plasma ADAMTS13 levels/activity and the risk of ischemic stroke and outcomes of recanalization therapies: a systematic review and meta-analysis.Frontiers in neurology · 2025Pooled it
- The effects of coagulation factors on the risk of endometriosis: a Mendelian randomization study.BMC medicine · 2023Pooled it
- The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: key findings at enrollment until 2017.Haematologica · 2019Trial
- ADAMTS13 and Von Willebrand factor in patients undergoing hemodialysis.Journal of thrombosis and thrombolysis · 2012Trial
- A mutant complement factor H (W1183R) enhances proteolytic cleavage of von Willebrand factor by ADAMTS-13 under shear.Journal of thrombosis and haemostasis : JTH · 2025Article
- Arginine Methylation by PRMT1 Affects ADAMTS13 Secretion and Enzymatic Activity.Arteriosclerosis, thrombosis, and vascular biology · 2025Article
- Novel mechanisms of action of emerging therapies of hereditary thrombotic thrombocytopenic purpura.Expert review of hematology · 2024Review
- Targeting neutrophil extracellular trap accumulation under flow in patients with immune-mediated thrombotic thrombocytopenic purpura.Blood advances · 2024Article
- Cathepsin K deficiency prevented stress-related thrombosis in a mouse FeClCellular and molecular life sciences : CMLS · 2024Article
- Dynamic Assessment of Plasma von Willebrand Factor and ADAMTS13 Predicts Mortality in Hospitalized Patients with SARS-CoV-2 Infection.Journal of clinical medicine · 2023Article
- ADAMTS-13 and HMGB1-induced oxidative stress in Taenia multiceps-infected animals.Scientific reports · 2023Article
- ADAMTS13 Biomarkers in Management of Immune Thrombotic Thrombocytopenic Purpura.Archives of pathology & laboratory medicine · 2023Review
- ADAMTS13 protease or lack of von Willebrand factor protects irradiation and melanoma-induced thrombotic microangiopathy in zebrafish.Journal of thrombosis and haemostasis : JTH · 2022Article
- Contribution of ADAMTS13-independent VWF regulation in sickle cell disease.Journal of thrombosis and haemostasis : JTH · 2022Article
- TTP: From empiricism for an enigmatic disease to targeted molecular therapies.British journal of haematology · 2022Review
- Association between ADAMTS13 deficiency and cardiovascular events in chronic hemodialysis patients.Scientific reports · 2021Observational
- Shedding Light on the Possible Link between ADAMTS13 and Vaccine-Induced Thrombotic Thrombocytopenia.Cells · 2021Article
- Novel mutations in ADAMTS13 CUB domains cause abnormal pre-mRNA splicing and defective secretion of ADAMTS13.Journal of cellular and molecular medicine · 2020Article
- Low Plasma ADAMTS13 Activity Is Associated with Coagulopathy, Endothelial Cell Damage and Mortality after Severe Paediatric Trauma.Thrombosis and haemostasis · 2018Article
- Role of calcium in regulating the intra- and extracellular cleavage of von Willebrand factor by the protease ADAMTS13.Blood advances · 2017Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
ADAMTS13 biosynthesis appeared to occur mainly in hepatic stellate cells, but detection of ADAMTS13 mRNA in many other tissues suggests that vascular endothelium may also produce ADAMTS13. We showed that ADAMTS13 mRNA and protein were detectable in human umbilical vein endothelial cells, aortic endothelial cells, and endothelium-derived cell line (ECV304). ADAMTS13 in cell lysate or serum-free conditioned medium cleaved von Willebrand factor (VWF) specifically. ADAMTS13 and VWF were localized to the distinct compartments of endothelial cells. Moreover, ADAMTS13 was preferentially sorted into apical domain of ECV304 and Madin-Darby canine kidney (MDCK) cells. Apical sorting of ADAMTS13 depended on the CUB domains and their association with lipid rafts. A mutation in the second CUB domain of ADAMTS13 (4143-4144insA), naturally occurring in patients with inherited thrombotic thrombocytopenic purpura, resulted in a significant reduction of ADAMTS13 secretion and a reversal of its polarity in MDCK cells. These data demonstrated that ADAMTS13 is synthesized and secreted from endothelial cells; the apically secreted ADAMTS13 from endothelial cells may contribute significantly to plasma ADAMTS13 proteases. The data also suggest a critical role of the CUB domains and a novel cargo-selective mechanism for apical sorting of a soluble ADAMTS protease in polarized cells.
Indexed as
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What OpenQuestion holds
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.