ArticleJournal of thrombosis and haemostasis : JTH2023
Coagulation factor VIII regulates von Willebrand factor homeostasis invivo.
Article in Journal of thrombosis and haemostasis : JTH, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed, 6 citations in OpenAlex.
- A Vortex-Shear-Based Assay for Cleavage of Multimeric VWF by ADAMTS13.Methods in molecular biology (Clifton, N.J.) · 2026Article
- Optical-Tweezer Single-Molecule Assay for ADAMTS13-Mediated Proteolysis of the von Willebrand Factor A2 Domain.Methods in molecular biology (Clifton, N.J.) · 2026Article
- Impact of ABO Blood Group on Bleeding and Clotting Time Among Healthy North Indian Males: A Pilot Study.Cureus · 2025Article
- Novel mechanisms of action of emerging therapies of hereditary thrombotic thrombocytopenic purpura.Expert review of hematology · 2024Review
Corrections and comments
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundCoagulation factor VIII (FVIII) and von Willebrand factor (VWF) circulate as a noncovalent complex, but each has its distinct functions. Binding of FVIII to VWF results in a prolongation of FVIII's half-life in circulation and modulates FVIII's immunogenicity during hemophilia therapy. However, the biological effect of FVIII and VWF interaction on VWF homeostasis is not fully understood.
objectivesTo determine the effect of FVIII in VWF proteolysis and homeostasis in vivo.
methodsMouse models, recombinant FVIII infusion, and patients with hemophilia A on a high dose FVIII for immune tolerance induction therapy or emicizumab for bleeding symptoms were included to address this question.
resultsAn intravenous infusion of a recombinant B-domain less FVIII (BDD-FVIII) (40 and 160 μg/kg) into wild-type mice significantly reduced plasma VWF multimer sizes and its antigen levels; an infusion of a high but not low dose of BDD-FVIII into Adamts13
conclusionOur results demonstrate the pivotal role of FVIII as a cofactor regulating VWF proteolysis and homeostasis under various (patho)physiological conditions.
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