ArticleBlood2023
C1 inhibitor deficiency enhances contact pathway-mediated activation of coagulation and venous thrombosis.
Article in Blood, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed, 31 citations in OpenAlex.
- Prevalence and impact of sleep disorders in patients with hereditary angioedema: A multicenter cross-sectional study in Latin America.Sleep & breathing = Schlaf & Atmung · 2026Article
- Bradykinin contributes to vasogenic edema in murine experimental cerebral malaria.The Journal of clinical investigation · 2026Article
- Potential Role of Contact Pathway Factors in Catheter-Related Thrombosis: Emerging Evidence and Therapeutic Strategies.Biomolecules · 2026Review
- Neutrophil CD14 is a driver and a therapeutic target for deep vein thrombosis.Blood advances · 2026Article
- Factor XII in Thrombosis and Thromboinflammation: From Molecular Biology to Clinical Translation.International journal of molecular sciences · 2026Review
- Population-scale analysis reveals inherited C1-inhibitor deficiency is a polyphenotypic thrombotic disorder.Blood advances · 2026Article
- Exploring the role of C1-inhibitor in thrombotic diseases.Blood advances · 2026Article
- Therapeutic Potential of C1-Inhibitor in Vascular Diseases and Beyond.Arteriosclerosis, thrombosis, and vascular biology · 2026Review
- Complement in acute kidney injury: a convergent pathogenic pathway in multifactorial renal damage.Frontiers in immunology · 2026Review
- Associations of hereditary angioedema attacks with intestinal permeability and inflammatory cytokines.Frontiers in immunology · 2026Article
- Endothelial barrier disorder in hereditary angioedema: molecular mechanisms and therapeutic implications.Frontiers in immunology · 2026Review
- Review
- Coagulation factor XII haploinsufficiency is protective against venous thromboembolism in a population-scale multidimensional analysis.Nature communications · 2025Article
- NET Proteomic Profiling Reveals New Pathways Potentially Implicated in Dendritic Cell-Mediated Inflammation in DADA2 Patients.Journal of clinical immunology · 2025Article
- Hereditary Angioedema and Venous Thromboembolism: Where There's Smoke, There's Fire.Seminars in thrombosis and hemostasis · 2025Review
- Enhancement of plasma kallikrein specificity of antitrypsin variants identified by phage display and partial reversion.BMC biotechnology · 2025Article
- The Basic Principles of Pathophysiology of Venous Thrombosis.International journal of molecular sciences · 2024Review
- Factor XII Structure-Function Relationships.Seminars in thrombosis and hemostasis · 2024Review
- Uncovering a novel SERPING1 pathogenic variant: insights into the aggregation of C1-INH in hereditary angioedema.Orphanet journal of rare diseases · 2024Article
- Illustrated State-of-the-Art Capsules of the ISTH 2024 Congress.Research and practice in thrombosis and haemostasis · 2024Article
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Authors and funding
16 authors at 5 institutions in 4 countries.
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Abstract
C1 inhibitor (C1INH) is a multifunctional serine protease inhibitor that functions as a major negative regulator of several biological pathways, including the contact pathway of blood coagulation. In humans, congenital C1INH deficiency results in a rare episodic bradykinin-mediated swelling disorder called hereditary angioedema (HAE). Patients with C1INH deficiency-associated HAE (C1INH-HAE) have increased circulating markers of activation of coagulation. Furthermore, we recently reported that patients with C1INH-HAE had a moderate but significant increased risk of venous thromboembolism. To further investigate the impact of C1INH deficiency on activation of coagulation and thrombosis, we conducted studies using patient samples and mouse models. Plasmas from patients with C1INH-HAE had significantly increased contact pathway-mediated thrombin generation. C1INH-deficient mice, which have been used as a model of C1INH-HAE, had significantly increased baseline circulating levels of prothrombin fragment 1+2 and thrombin-antithrombin complexes. In addition, whole blood from C1INH-deficient mice supported significantly increased contact pathway-mediated thrombin generation. Importantly, C1INH-deficient mice exhibited significantly enhanced venous, but not arterial, thrombus formation. Furthermore, purified human C1INH normalized contact pathway-mediated thrombin generation and venous thrombosis in C1INH-deficient mice. These findings highlight a key role for endogenous C1INH as a negative regulator of contact pathway-mediated coagulation in humans and mice. Further, this work identifies endogenous C1INH as an important negative regulator of venous thrombus formation in mice, complementing the phenotype associated with C1INH-HAE.
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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.