ArticleBlood2020
EPCR deficiency or function-blocking antibody protects against joint bleeding-induced pathology in hemophilia mice.
Article in Blood, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 24 citations in OpenAlex.
- Characterization of a mouse model to study mechanisms of hemophilia A pain.Blood advances · 2026Article
- Lysine lactylation in diseases: beyond histone lactylation.Cell death & disease · 2025Review
- Exosome-loaded tannic acid-thioctic acid hydrogel enhances wound healing in coagulation disorders.Materials today. Bio · 2025Article
- Endothelial Protein C Receptor: A Multifunctional Mediator in the Pathophysiology of Rheumatoid Arthritis.Cells · 2025Review
- Novel injectable adhesive hydrogel loaded with exosomes for holistic repair of hemophilic articular cartilage defect.Bioactive materials · 2024Article
- Endothelial Protein C Receptor and Its Impact on Rheumatic Disease.Journal of clinical medicine · 2024Review
- EPCR deficiency ameliorates inflammatory arthritis in mice by suppressing the activation and migration of T cells and dendritic cells.Rheumatology (Oxford, England) · 2024Article
- Endothelial Protein C Receptor and 3K3A-Activated Protein C Protect Mice from Allergic Contact Dermatitis in a Contact Hypersensitivity Model.International journal of molecular sciences · 2024Article
- Exploring the association between circulating endothelial protein C receptor and disease activity of rheumatoid arthritis in a pilot study.Rheumatology advances in practice · 2024Article
- Hematopoietic stem cells undergo a lymphoid to myeloid switch in early stages of emergency granulopoiesis.The EMBO journal · 2023Article
- The Vascular Endothelium and Coagulation: Homeostasis, Disease, and Treatment, with a Focus on the Von Willebrand Factor and Factors VIII and V.International journal of molecular sciences · 2022Review
- Blocking human protein C anticoagulant activity improves clotting defects of hemophilia mice expressing human protein C.Blood advances · 2022Article
- Article
- Isoorientin suppresses sepsis-induced acute lung injury in mice by activating an EPCR-dependent JAK2/STAT3 pathway.Journal of molecular histology · 2022Article
- Natural anticoagulants: A missing link in mild to moderate bleeding tendencies.Haemophilia : the official journal of the World Federation of Hemophilia · 2021Review
- EPCR knockout: inflaming the discussion.Blood · 2020Article
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4 authors at 2 institutions in 1 country.
Funding
Abstract
We recently showed that clotting factor VIIa (FVIIa) binding to endothelial cell protein C receptor (EPCR) induces anti-inflammatory signaling and protects vascular barrier integrity. Inflammation and vascular permeability are thought to be major contributors to the development of hemophilic arthropathy following hemarthrosis. The present study was designed to investigate the potential influence of FVIIa interaction with EPCR in the pathogenesis of hemophilic arthropathy and its treatment with recombinant FVIIa (rFVIIa). For this, we first generated hemophilia A (FVIII-/-) mice lacking EPCR (EPCR-/-FVIII-/-) or overexpressing EPCR (EPCR++ FVIII-/-). Joint bleeding was induced in FVIII-/-, EPCR-/-FVIII-/-, and EPCR++FVIII-/- mice by needle puncture injury. Hemophilic synovitis was evaluated by monitoring joint bleeding, change in joint diameter, and histopathological analysis of joint tissue sections. EPCR deficiency in FVIII-/- mice significantly reduced the severity of hemophilic synovitis. EPCR deficiency attenuated the elaboration of interleukin-6, infiltration of macrophages, and neoangiogenesis in the synovium following hemarthrosis. A single dose of rFVIIa was sufficient to fully prevent the development of milder hemophilic synovitis in EPCR-/-FVIII-/- mice. The development of hemophilic arthropathy in EPCR-overexpressing FVIII-/- mice did not significantly differ from that of FVIII-/- mice, and 3 doses of rFVIIa partly protected against hemophilic synovitis in these mice. Consistent with the data that EPCR deficiency protects against developing hemophilic arthropathy, administration of a single dose of EPCR-blocking monoclonal antibodies markedly reduced hemophilic synovitis in FVIII-/- mice subjected to joint bleeding. The present data indicate that EPCR could be an attractive new target to prevent joint damage in hemophilia patients.
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