ArticleJournal of the American Society of Nephrology : JASN2011
The development of atypical hemolytic uremic syndrome depends on complement C5.
Article in Journal of the American Society of Nephrology : JASN, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 44 papers.
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Who cites it
44 citing papers in PubMed, 114 citations in OpenAlex.
- Trial
- Common hub genes in Uremia and Kidney Renal Clear Cell Carcinoma (KIRC): their role in KIRC pathogenesis through activation of the citrate cycle pathway.BMC nephrology · 2025Article
- Enigmatic Roles of Complement Anaphylatoxin Signaling in Health and Disease.Immune network · 2025Review
- Complement abnormality predisposes to the development of malignant hypertension-associated thrombotic microangiopathy disease.Clinical kidney journal · 2025Article
- Design and Rationale of the APPELHUS Phase 3 Open-Label Study of Factor B Inhibitor Iptacopan for Atypical Hemolytic Uremic Syndrome.Kidney international reports · 2023Article
- The Rational Use of Complement Inhibitors in Kidney Diseases.Kidney international reports · 2022Review
- Patient-specific iPSC-derived endothelial cells reveal aberrant p38 MAPK signaling in atypical hemolytic uremic syndrome.Stem cell reports · 2021Article
- The Syndromes of Thrombotic Microangiopathy: A Critical Appraisal on Complement Dysregulation.Journal of clinical medicine · 2021Review
- Alternative complement pathway activation in thrombotic microangiopathy associated with lupus nephritis.Clinical rheumatology · 2021Article
- Gain-of-function factor H-related 5 protein impairs glomerular complement regulation resulting in kidney damage.Proceedings of the National Academy of Sciences of the United States of America · 2021Article
- Complement activity is regulated in C3 glomerulopathy by IgG-factor H fusion proteins with and without properdin targeting domains.Kidney international · 2021Article
- COVID-19: A review of therapeutic strategies and vaccine candidates.Clinical immunology (Orlando, Fla.) · 2021Review
- Hereditary Deficiency of the Second Component of Complement: Early Diagnosis and 21-Year Follow-Up of a Family.Medicina (Kaunas, Lithuania) · 2020Article
- Hyperfunctional complement C3 promotes C5-dependent atypical hemolytic uremic syndrome in mice.The Journal of clinical investigation · 2019Article
- Endothelium structure and function in kidney health and disease.Nature reviews. Nephrology · 2019Review
- Mother and Child Reunion in "Hypertensive" End-Stage Renal Disease: Will They Complement Each Other?Nephron · 2019Article
- C5b9 Formation on Endothelial Cells Reflects Complement Defects among Patients with Renal Thrombotic Microangiopathy and Severe Hypertension.Journal of the American Society of Nephrology : JASN · 2018Article
- A case of atypical hemolytic uremic syndrome in a second renal transplant.Journal of nephrology · 2018Article
- Clinical and Complement Long-Term Follow-Up of a Pediatric Patient with C3 Mutation-Related Atypical Hemolytic Uremic Syndrome.Case reports in nephrology · 2018Article
- A Single-Domain Antibody Targeting Complement Component C5 Acts as a Selective Inhibitor of the Terminal Pathway of the Complement System and Thus Functionally Mimicks the C-Terminal Domain of theFrontiers in immunology · 2018Article
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Authors and funding
8 authors at 3 institutions in 2 countries.
Funding
Abstract
Gene variants in the alternative pathway of the complement system strongly associate with atypical hemolytic uremic syndrome (aHUS), presumably by predisposing to increased complement activation within the kidney. Complement factor H (CFH) is the major regulator of complement activation through the alternative pathway. Factor H-deficient mice transgenically expressing a mutant CFH protein (Cfh(-/-).FHΔ16-20) that functionally mimics the CFH mutations reported in aHUS patients spontaneously develop thrombotic microangiopathy. To investigate the role of complement C5 activation in this aHUS model, we generated C5-deficient Cfh(-/-).FHΔ16-20 mice. Both C5-sufficient and C5-deficient Cfh(-/-).FHΔ16-20 mice had abnormal C3 deposition within the kidney, but spontaneous aHUS did not develop in any of the C5-deficient mice. Furthermore, although Cfh(-/-).FHΔ16-20 animals demonstrated marked hypersensitivity to experimentally triggered renal injury, animals with concomitant C5 deficiency did not. These data demonstrate a critical role for C5 activation in both spontaneous aHUS and experimentally triggered renal injury in animals with defective complement factor H function. This study provides a rationale to investigate therapeutic inhibition of C5 in human aHUS.
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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.