ArticlePediatric nephrology (Berlin, Germany)2019
Immunological features and functional analysis of anti-CFH autoantibodies in patients with atypical hemolytic uremic syndrome.
Article in Pediatric nephrology (Berlin, Germany), 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 19 citations in OpenAlex.
- Comprehensive genetic analysis and genotype-phenotype correlations in pediatric patients with atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2025Article
- Protein-losing enteropathy as a new phenotype in atypical hemolytic uremic syndrome caused by CD46 gene mutation.Pediatric nephrology (Berlin, Germany) · 2024Review
- Anti-complement factor H (CFH) autoantibodies could delay pristane-induced lupus nephritis.Immunologic research · 2023Article
- Underlying Genetics of aHUS: Which Connection with Outcome and Treatment Discontinuation?International journal of molecular sciences · 2023Review
- Anti-factor H antibody and its role in atypical hemolytic uremic syndrome.Frontiers in immunology · 2022Article
- Review
- Patient-specific iPSC-derived endothelial cells reveal aberrant p38 MAPK signaling in atypical hemolytic uremic syndrome.Stem cell reports · 2021Article
- Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic Syndrome.International journal of nephrology · 2021Article
- Case Report: Severe Complement-Mediated Thrombotic Microangiopathy in IgG4-Related Disease Secondary to Anti-Factor H IgG4 Autoantibodies.Frontiers in immunology · 2020Article
- Factor H Autoantibodies and Complement-Mediated Diseases.Frontiers in immunology · 2020Article
- Clinical and Immunological Profile of Anti-factor H Antibody Associated Atypical Hemolytic Uremic Syndrome: A Nationwide Database.Frontiers in immunology · 2019Article
- Rare Functional Variants in Complement Genes and Anti-FH Autoantibodies-Associated aHUS.Frontiers in immunology · 2019Article
- Immunological Characteristics of Chinese Adult Patients with Anti-Complement Factor H Autoantibodies-Associated Complement-Mediated Thrombotic Microangiopathy.Kidney diseases (Basel, Switzerland)Article
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Authors and funding
12 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveAtypical hemolytic uremic syndrome (aHUS) is associated with defective complement regulation. Anti-complement factor H (CFH) antibodies were thought to participate in the pathogenesis of aHUS. The aim of this study was to address the functions and properties of CFH autoantibodies in a Chinese Han cohort of aHUS patients.
methodsThirty-six anti-CFH antibody-positive aHUS patients at the acute phase of the disease were involved in this study. Clinical data of the patients were collected. Anti-CFH immunoglobulin G (IgG) subclasses and antibody isotypes were detected by ELISA. Epitope mapping was performed using recombinant CFH fragments (SCRs 1-4, SCR 7, SCRs 11-14, and SCRs 19-20). Purified IgG from plasma from seven patients were used for functional analyses.
resultsAll patients presented with the classic triad of HUS. The anti-CFH autoantibodies mostly bound to the SCRs 19-20 domains of CFH but not the SCRs 1-4 domains. CFI cofactor activity was not disturbed by the anti-CFH antibody in any of the seven patients. Purified IgG interfered with the binding of CFH to C3b and CFH-mediated sheep erythrocyte protection in all seven patients. IgG from 4/5 (80%) patients tested inhibited the binding of CFH to glomerular endothelial cells.
conclusionsOur study suggests that the properties of CFH antibodies from patients with aHUS, including the recognition of SCRs and IgG subclasses, can influence and impair the biological role of CFH and therefore contribute to aHUS susceptibility.
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