ArticleProceedings of the National Academy of Sciences of the United States of America2022
Endothelial VWF is critical for the pathogenesis of vaso-occlusive episode in a mouse model of sickle cell disease.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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15 citing papers in PubMed, 22 citations in OpenAlex.
- Review
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- Allele-selective von Willebrand factor silencing.Research and practice in thrombosis and haemostasis · 2026Article
- Emerging pathways in thromboinflammation of sickle cell disease: novel findings in disease pathogenesis.Research and practice in thrombosis and haemostasis · 2025Review
- Blood perfusion through ventricular assist devices induces erythrocytes to interact with leukocytes.Biochemistry and biophysics reports · 2025Article
- From early development to maturity: a phenotypic analysis of the Townes sickle cell disease mice.Biology open · 2025Article
- Novel functions for von Willebrand factor.Blood · 2024Review
- Biased agonism of protease-activated receptor-1 regulates thromboinflammation in murine sickle cell disease.Blood advances · 2024Article
- Article
- Editorial: Inflammation, the link between venous and arterial thrombosis.Frontiers in cardiovascular medicine · 2024Article
- Mouse models of sickle cell disease: Imperfect and yet very informative.Blood cells, molecules & diseases · 2024Review
- VWF-ADAMTS13 axis dysfunction in children with sickle cell disease treated with hydroxycarbamide vs blood transfusion.Blood advances · 2023Article
- Factor XII contributes to thrombotic complications and vaso-occlusion in sickle cell disease.Blood · 2023Article
- Targeting the von Willebrand Factor-ADAMTS-13 axis in sickle cell disease.Journal of thrombosis and haemostasis : JTH · 2023Article
- Altered VWF:ADAMTS13 homeostasis is a target for therapeutic intervention in sickle cell disease.Proceedings of the National Academy of Sciences of the United States of America · 2022Article
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12 authors at 2 institutions in 1 country.
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Abstract
Vaso-occlusive episode (VOE) is a common and critical complication of sickle cell disease (SCD). Its pathogenesis is incompletely understood. von Willebrand factor (VWF), a multimeric plasma hemostatic protein synthesized and secreted by endothelial cells and platelets, is increased during a VOE. However, whether and how VWF contributes to the pathogenesis of VOE is not fully understood. In this study, we found increased VWF levels during tumor necrosis factor (TNF)-induced VOE in a humanized mouse model of SCD. Deletion of endothelial VWF decreased hemolysis, vascular occlusion, and organ damage caused by TNF-induced VOE in SCD mice. Moreover, administering ADAMTS13, the VWF-cleaving plasma protease, reduced plasma VWF levels, decreased inflammation and vaso-occlusion, and alleviated organ damage during VOE. These data suggest that promoting VWF cleavage via ADAMTS13 may be an effective treatment for reducing hemolysis, inflammation, and vaso-occlusion during VOE.
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