ArticleThe EMBO journal2006
Structure of complement factor H carboxyl-terminus reveals molecular basis of atypical haemolytic uremic syndrome.
Article in The EMBO journal, 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 63 papers.
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Who cites it
63 citing papers in PubMed, 157 citations in OpenAlex.
- Y402H polymorphism of complement factor H affects binding affinity to C-reactive protein.Journal of immunology (Baltimore, Md. : 1950) · 2007Trial
- The C-terminal domain of Staphylococcus aureus Efb recruits FHR-2 to C3b, synergistically inhibiting the terminal complement pathway.Journal of immunology (Baltimore, Md. : 1950) · 2026Article
- Complement therapeutic factor H-IgG proteins as pre-exposure prophylaxes against Lyme borreliae infections.Journal of immunology (Baltimore, Md. : 1950) · 2025Article
- Complement therapeutic Factor H-IgG proteins as pre-exposure prophylaxes against Lyme borreliae infections.bioRxiv : the preprint server for biology · 2024Article
- Mechanism of Borrelia immune evasion by FhbA-related proteins.PLoS pathogens · 2022Article
- An optimized Factor H-Fc fusion protein against multidrug-resistantFrontiers in immunology · 2022Article
- Neonatal thrombotic microangiopathy secondary to factor I variant with Hirschsprung disease.Journal of nephrology · 2021Article
- The solution structure of the complement deregulator FHR5 reveals a compact dimer and provides new insights into CFHR5 nephropathy.The Journal of biological chemistry · 2020Article
- Beyond Panel-Based Testing: Exome Analysis Increases Sensitivity for Diagnosis of Genetic Kidney Disease.Kidney360 · 2020Article
- Characterization of Binding Properties of Individual Functional Sites of Human Complement Factor H.Frontiers in immunology · 2020Article
- Development of Complement Factor H-Based Immunotherapeutic Molecules in Tobacco Plants Against Multidrug-ResistantFrontiers in immunology · 2020Article
- The Human Platelet as an Innate Immune Cell: Interactions Between Activated Platelets and the Complement System.Frontiers in immunology · 2019Review
- Two distinct conformations of factor H regulate discrete complement-binding functions in the fluid phase and at cell surfaces.The Journal of biological chemistry · 2018Article
- An Engineered Complement Factor H Construct for Treatment of C3 Glomerulopathy.Journal of the American Society of Nephrology : JASN · 2018Article
- Analysis of Linear Antibody Epitopes on Factor H and CFHR1 Using Sera of Patients with Autoimmune Atypical Hemolytic Uremic Syndrome.Frontiers in immunology · 2017Article
- Crystal structure of a tripartite complex between C3dg, C-terminal domains of factor H and OspE of Borrelia burgdorferi.PloS one · 2017Article
- Staphylococcal protein Ecb impairs complement receptor-1 mediated recognition of opsonized bacteria.PloS one · 2017Article
- Protection of host cells by complement regulators.Immunological reviews · 2016Review
- Properdin: a tightly regulated critical inflammatory modulator.Immunological reviews · 2016Review
- Mutations in Complement Factor H Impair Alternative Pathway Regulation on Mouse Glomerular Endothelial Cells in Vitro.The Journal of biological chemistry · 2016Article
3 more citing papers are in PubMed but not listed here.
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Factor H (FH) is the key regulator of the alternative pathway of complement. The carboxyl-terminal domains 19-20 of FH interact with the major opsonin C3b, glycosaminoglycans, and endothelial cells. Mutations within this area are associated with atypical haemolytic uremic syndrome (aHUS), a disease characterized by damage to endothelial cells, erythrocytes, and kidney glomeruli. The structure of recombinant FH19-20, solved at 1.8 A by X-ray crystallography, reveals that the short consensus repeat domain 20 contains, unusually, a short alpha-helix, and a patch of basic residues at its base. Most aHUS-associated mutations either destabilize the structure or cluster in a unique region on the surface of FH20. This region is close to, but distinct from, the primary heparin-binding patch of basic residues. By mutating five residues in this region, we show that it is involved, not in heparin, but in C3b binding. Therefore, the majority of the aHUS-associated mutations on the surface of FH19-20 interfere with the interaction between FH and C3b. This obviously leads to impaired control of complement attack on plasma-exposed cell surfaces in 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.