ArticlePloS one2013
Human factor H-related protein 2 (CFHR2) regulates complement activation.
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 48 papers.
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Who cites it
48 citing papers in PubMed, 68 citations in OpenAlex.
- Plasma Proteomic Study in Pulmonary Arterial Hypertension Associated with Congenital Heart Diseases.Scientific reports · 2016Trial
- Decoding the Factor H-Related Proteins: Gatekeepers of Complement Dysregulation in AMD.European journal of immunology · 2026Review
- Urine Proteomic Profiling at Admission Reveals Complement Biomarkers Linked to Alcohol-Associated Liver Disease.Alcohol, clinical & experimental research · 2026Article
- Urinary CFHR2 as a Biomarker for Early Diagnosis and Disease Progression Prediction in Diabetic Kidney Disease.Current molecular medicine · 2026Article
- Hepatic steatosis in postmenopausal women is characterized by distinct serum extracellular vesicle proteomic signatures.BMC medicine · 2025Article
- Analysis of human factor H-related gene and protein expressed in rheumatoid arthritis synovium identifies a novel mechanism promoting dysregulated complement pathway activation.Scientific reports · 2025Article
- Elevated Plasma Complement Factors in CRB1-Associated Inherited Retinal Dystrophies.Investigative ophthalmology & visual science · 2025Article
- The human factor H protein family - an update.Frontiers in immunology · 2024Review
- Association of Risk Variants in the CFH Gene With Elevated Levels of Coagulation and Complement Factors in Idiopathic Multifocal Choroiditis.JAMA ophthalmology · 2023Article
- The Factor H protein family: The switchers of the complement alternative pathway.Immunological reviews · 2023Review
- Factor H related proteins modulate complement activation on kidney cells.Kidney international · 2022Article
- Role of Axl in target organ inflammation and damage due to hypertensive aortic remodeling.American journal of physiology. Heart and circulatory physiology · 2022Article
- Complement Factor H and Related Proteins as Markers of Cardiovascular Risk in Pediatric Chronic Kidney Disease.Biomedicines · 2022Article
- Alterations in Self-Aggregating Neuropeptides in Cerebrospinal Fluid of Patients with Parkinsonian Disorders.Journal of Parkinson's disease · 2022Article
- Case report: Novel FHR2 variants in atypical Hemolytic Uremic Syndrome: A case study of a translational medicine approach in renal transplantation.Frontiers in immunology · 2022Article
- A Family Affair: Addressing the Challenges of Factor H and the Related Proteins.Frontiers in immunology · 2021Review
- Screening and identification of serum biomarkers of osteoarticular tuberculosis based on mass spectrometry.Journal of clinical laboratory analysis · 2020Article
- Proteome Multimarker Panel With Multiple Reaction Monitoring-Mass Spectrometry for Early Detection of Hepatocellular Carcinoma.Hepatology communications · 2020Article
- Article
- Deficiency of Mouse FHR-1 Homolog, FHR-E, Accelerates Sepsis, and Acute Kidney Injury Through Enhancing the LPS-Induced Alternative Complement Pathway.Frontiers in immunology · 2020Article
Corrections and comments
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Authors and funding
11 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Mutations and deletions within the human CFHR gene cluster on chromosome 1 are associated with diseases, such as dense deposit disease, CFHR nephropathy or age-related macular degeneration. Resulting mutant CFHR proteins can affect complement regulation. Here we identify human CFHR2 as a novel alternative pathway complement regulator that inhibits the C3 alternative pathway convertase and terminal pathway assembly. CFHR2 is composed of four short consensus repeat domains (SCRs). Two CFHR2 molecules form a dimer through their N-terminal SCRs, and each of the two C-terminal ends can bind C3b. C3b bound CFHR2 still allows C3 convertase formation but the CFHR2 bound convertases do not cleave the substrate C3. Interestingly CFHR2 hardly competes off factor H from C3b. Thus CFHR2 likely acts in concert with factor H, as CFHR2 inhibits convertases while simultaneously allowing factor H assisted degradation by factor I.
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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.