ArticleThe Journal of biological chemistry2017
Disease-linked mutations in factor H reveal pivotal role of cofactor activity in self-surface-selective regulation of complement activation.
Article in The Journal of biological chemistry, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 27 citations in OpenAlex.
- Truncated complement factor H Y402 gene therapy rescues C3 glomerulonephritis.Molecular therapy : the journal of the American Society of Gene Therapy · 2025Article
- An Evaluation of the Complement-Regulating Activities of Human Complement Factor H (FH) Variants Associated With Age-Related Macular Degeneration.Investigative ophthalmology & visual science · 2022Article
- Antibody recognition of complement factor H reveals a flexible loop involved in atypical hemolytic uremic syndrome pathogenesis.The Journal of biological chemistry · 2022Article
- Multifaceted Activities of Seven Nanobodies against Complement C4b.Journal of immunology (Baltimore, Md. : 1950) · 2022Article
- Two human antibodies to a meningococcal serogroup B vaccine antigen enhance binding of complement Factor H by stabilizing the Factor H binding site.PLoS pathogens · 2021Article
- Homodimeric Minimal Factor H:Frontiers in immunology · 2021Article
- Murine Factor H Co-Produced in Yeast With Protein Disulfide Isomerase Ameliorated C3 Dysregulation in Factor H-Deficient Mice.Frontiers in immunology · 2021Article
- New insights into the pathogenesis of Streptococcus pneumoniae-associated hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2020Review
- Common variants of fetal and maternal complement genes in preeclampsia: pregnancy specific complotype.Scientific reports · 2020Article
- Functional Characterization of Rare Genetic Variants in the N-Terminus of Complement Factor H in aHUS, C3G, and AMD.Frontiers in immunology · 2020Article
- Combining SPR with atomic-force microscopy enables single-molecule insights into activation and suppression of the complement cascade.The Journal of biological chemistry · 2019Article
- An Engineered Complement Factor H Construct for Treatment of C3 Glomerulopathy.Journal of the American Society of Nephrology : JASN · 2018Article
- Utilization of Staphylococcal Immune Evasion Protein Sbi as a Novel Vaccine Adjuvant.Frontiers in immunology · 2018Article
- Complement Factor H Inhibits Anti-Neutrophil Cytoplasmic Autoantibody-Induced Neutrophil Activation by Interacting With Neutrophils.Frontiers in immunology · 2018Article
- Functional Characterization of the Disease-Associated N-Terminal Complement Factor H Mutation W198R.Frontiers in immunology · 2017Article
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
Abstract
Spontaneous activation enables the complement system to respond very rapidly to diverse threats. This activation is efficiently suppressed by complement factor H (CFH) on self-surfaces but not on foreign surfaces. The surface selectivity of CFH, a soluble protein containing 20 complement-control protein modules (CCPs 1-20), may be compromised by disease-linked mutations. However, which of the several functions of CFH drives this self-surface selectivity remains unknown. To address this, we expressed human CFH mutants in
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