ArticleBlood2017
Murine systemic thrombophilia and hemolytic uremic syndrome from a factor H point mutation.
Article in Blood, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 45 papers.
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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.
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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.
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Who cites it
45 citing papers in PubMed, 56 citations in OpenAlex.
- ASSOCIATION BETWEEN ORAL IRON SUPPLEMENTATION AND RETINAL OR SUBRETINAL HEMORRHAGE IN THE COMPARISON OF AGE-RELATED MACULAR DEGENERATION TREATMENT TRIALS.Retina (Philadelphia, Pa.) · 2019Trial
- Neutrophil Extracellular Traps Aggravate Injury in Complement-Mediated Thrombotic Microangiopathy.Journal of the American Society of Nephrology : JASN · 2026Article
- Complement Factor H as a Novel Predictive Biomarker for Deep Vein Thrombosis Recurrence: A Prospective Cohort Study.Journal of thrombosis and thrombolysis · 2026Article
- Serum complement C3 as a candidate biomarker for monitoring progression from chronic kidney disease to kidney failure.International urology and nephrology · 2026Article
- Treatment of atypical hemolytic uremic syndrome and C3 glomerulopathy in mice by hepatic expression of factor D.Blood advances · 2026Article
- Crovalimab Rescue Therapy in a Case With Genetic Complement Mediated Thrombotic Microangiopathy.Kidney medicine · 2026Article
- Properdin deficiency or anti-properdin treatment ameliorates disease in the C3 gain-of-function mouse model of atypical haemolytic uraemic syndrome.Frontiers in immunology · 2026Article
- Friend or foe: assessing the value of animal models for facilitating clinical breakthroughs in complement research.The Journal of clinical investigation · 2025Review
- 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
- Improved therapeutic efficacy of a bifunctional anti-C5 mAb-FH SCR1-5 fusion protein over anti-C5 mAb in an accelerated mouse model of C3 glomerulopathy.ImmunoHorizons · 2025Article
- Lactobacillus casei Cell Wall Extract and Production of Galactose-Deficient IgA1 in a Humanized IGHA1 Mouse Model.Journal of the American Society of Nephrology : JASN · 2025Article
- Genetic variants contribute to modulation of renal function in patients with immune thrombotic thrombocytopenic purpura.Blood vessels, thrombosis & hemostasis · 2024Article
- Role of complement factor D in cardiovascular and metabolic diseases.Frontiers in immunology · 2024Review
- Heterozygous mutations in factor H aggravate pathological damage in a stable IgA deposition model induced byFrontiers in immunology · 2024Article
- Immune thrombotic thrombocytopenic purpura: pathogenesis and novel therapies: a narrative review.Annals of blood · 2023Article
- Association of Risk Variants in the CFH Gene With Elevated Levels of Coagulation and Complement Factors in Idiopathic Multifocal Choroiditis.JAMA ophthalmology · 2023Article
- Complement inhibition can decrease the haemostatic response in a microvascular bleeding model at multiple levels.Frontiers in immunology · 2023Article
- Contribution of animal models to the mechanistic understanding of Alternative Pathway and Amplification Loop (AP/AL)-driven Complement-mediated Diseases.Immunological reviews · 2023Review
- Complement system-related genes in stomach adenocarcinoma: Prognostic signature, immune landscape, and drug resistance.Frontiers in genetics · 2022Article
- A Simple, Robust, and Cost-effective Method for Genotyping Small-scale Mutations.Journal of clinical and translational pathology · 2022Article
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Authors and funding
18 authors at 5 institutions in 1 country.
Funding
Abstract
Complement plays a key role in host defense, but its dysregulation can cause autologous tissue injury. Complement activation is normally controlled by regulatory proteins, including factor H (FH) in plasma and membrane cofactor protein (MCP) on the cell surface. Mutations in FH and MCP are linked to atypical hemolytic uremic syndrome, a type of thrombotic microangiopathy (TMA) that causes renal failure. We describe here that disruption of FH function on the cell surface can also lead to disseminated complement-dependent macrovascular thrombosis. By gene targeting, we introduced a point mutation (W1206R) into murine FH that impaired its interaction with host cells but did not affect its plasma complement-regulating activity. Homozygous mutant mice carrying this mutation developed renal TMA as well as systemic thrombophilia involving large blood vessels in multiple organs, including liver, lung, spleen, and kidney. Approximately 30% of mutant mice displayed symptoms of stroke and ischemic retinopathy, and 48% died prematurely. Genetic deficiency of complement C3 and factor D prevented both the systemic thrombophilia and renal TMA phenotypes. These results demonstrate a causal relationship between complement dysregulation and systemic angiopathy and suggest that complement activation may contribute to various human thrombotic disorders involving both the micro- and macrovasculature.
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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.