{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T12:19:01Z","timestamp":1790252341042,"version":"4.1.0"},"reference-count":129,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2025,5,13]],"date-time":"2025-05-13T00:00:00Z","timestamp":1747094400000},"content-version":"vor","delay-in-days":1,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100004807","name":"DFG","doi-asserted-by":"publisher","award":["SFB 1192"],"award-info":[{"award-number":["SFB 1192"]}],"id":[{"id":"10.13039\/100004807","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,9,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Factor H controls proximal complement activation, and its dysfunction leads to diseases that often manifest in the kidney. Structural and functional analyses have identified 4 distinct functional segments: an N-terminal regulatory unit, a cell binding unit, a segment with combined low-affinity C3b and heparin sites, and a C-terminal recognition or sensor unit with overlapping C3b\/C3d and heparin sites. Three segments are linked to diseases. The regulatory segment is affected in C3 glomerulopathy and antineutrophil cytoplasmic antibody\u2013associated vasculitis. The second segment includes the Y402H polymorphism of age-related macular degeneration, is associated with different types of cancer, and is targeted by pathogens. The C-terminal sensor segment is involved in atypical hemolytic uremic syndrome, in FHR1:FHR3 deficient and autoantibody\u2013positive hemolytic uremic syndrome form and is exploited by pathogens. Factor H function is modulated by Factor H like protein 1 and FHR1, 2 plasma proteins that share segments with Factor H. This interplay is critical for fine-tuning local complement. Understanding Factor H\u2019s physiological role, as well as the impact of its absence, mutations, or autoantibody targeting, provides insights into disease mechanisms and provides opportunities for therapeutic intervention by using full-length Factor H, its fragments, or complement-modulatory compounds.<\/jats:p>","DOI":"10.1093\/jimmun\/vkaf065","type":"journal-article","created":{"date-parts":[[2025,5,13]],"date-time":"2025-05-13T00:34:24Z","timestamp":1747096464000},"page":"2150-2164","source":"Crossref","is-referenced-by-count":7,"title":["The 4 functional segments of Factor H: Role in physiological target recognition and contribution to disease"],"prefix":"10.1093","volume":"214","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6149-2411","authenticated-orcid":false,"given":"Peter F","family":"Zipfel","sequence":"first","affiliation":[{"name":"Department of Infection Biology, Leibniz Institute for Natural Product Research and Infection Biology , Jena,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karin","family":"Heidenreich","sequence":"additional","affiliation":[{"name":"Eleva GmbH , Freiburg,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,5,12]]},"reference":[{"issue":"Suppl 8","key":"2025093003514440300_vkaf065-B1","doi-asserted-by":"publisher","first-page":"I67","DOI":"10.1016\/j.vaccine.2008.11.015","article-title":"The complement fitness Factor: role in human diseases and for immune escape of pathogens, like pneumococci","volume":"26","author":"Zipfel","year":"2008","journal-title":"Vaccine"},{"key":"2025093003514440300_vkaf065-B2","doi-asserted-by":"publisher","first-page":"118","DOI":"10.1038\/s41577-023-00926-1","article-title":"A guide to complement biology, pathology and therapeutic opportunity","volume":"24","author":"Mastellos","year":"2024","journal-title":"Nat Rev Immunol"},{"key":"2025093003514440300_vkaf065-B3","doi-asserted-by":"publisher","first-page":"2166","DOI":"10.3389\/fimmu.2019.02166","article-title":"Complement inhibitors in clinical trials for glomerular diseases","volume":"10","author":"Zipfel","year":"2019","journal-title":"Front Immunol"},{"key":"2025093003514440300_vkaf065-B4","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1146\/annurev-med-042921-102405","article-title":"Expansion of anticomplement therapy indications from rare genetic disorders to common kidney diseases","volume":"75","author":"Miwa","year":"2024","journal-title":"Annu Rev Med"},{"key":"2025093003514440300_vkaf065-B5","doi-asserted-by":"publisher","first-page":"433","DOI":"10.1146\/annurev-immunol-032713-120154","article-title":"Complement and its receptors: new insights into human disease","volume":"32","author":"Holers","year":"2014","journal-title":"Annu Rev Immunol"},{"key":"2025093003514440300_vkaf065-B6","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1111\/imr.12474","article-title":"Evolution of the complement system: from defense of the single cell to guardian of the intravascular space","volume":"274","author":"Elvington","year":"2016","journal-title":"Immunol Rev"},{"key":"2025093003514440300_vkaf065-B7","doi-asserted-by":"publisher","first-page":"1106","DOI":"10.1016\/j.imbio.2012.07.008","article-title":"The tick-over theory revisited: is C3 a contact-activated protein?","volume":"217","author":"Nilsson","year":"2012","journal-title":"Immunobiology"},{"key":"2025093003514440300_vkaf065-B8","doi-asserted-by":"publisher","first-page":"120","DOI":"10.1111\/imr.13147","article-title":"C3-dependent effector functions of complement","volume":"313","author":"Zarantonello","year":"2023","journal-title":"Immunol Rev"},{"key":"2025093003514440300_vkaf065-B9","doi-asserted-by":"publisher","first-page":"426","DOI":"10.1038\/s41581-023-00704-1","article-title":"Complosome\u2014the intracellular complement system","volume":"19","author":"West","year":"2023","journal-title":"Nat Rev Nephrol"},{"key":"2025093003514440300_vkaf065-B10","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1055\/s-2001-15248","article-title":"Complement Factor H: physiology and pathophysiology","volume":"27","author":"Zipfel","year":"2001","journal-title":"Semin Thromb Hemost"},{"key":"2025093003514440300_vkaf065-B11","doi-asserted-by":"publisher","first-page":"2187","DOI":"10.1016\/j.molimm.2010.05.007","article-title":"Complement control protein factor: the good, the bad, and the inadequate","volume":"47","author":"Ferreira","year":"2010","journal-title":"Mol Immunol"},{"key":"2025093003514440300_vkaf065-B12","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1111\/j.1749-6632.1983.tb18116.x","article-title":"Initiation of the alternative complement pathway due to spontaneous hydrolysis of the thioester of C3","volume":"421","author":"Pangburn","year":"1983","journal-title":"Ann N Y Acad Sci"},{"key":"2025093003514440300_vkaf065-B13","doi-asserted-by":"publisher","first-page":"1566","DOI":"10.3390\/ijms25031566","article-title":"The lectin pathway of the complement system-activation, regulation, disease connections and interplay with other (proteolytic) systems","volume":"25","author":"Dob\u00f3","year":"2024","journal-title":"Int J Mol Sci"},{"key":"2025093003514440300_vkaf065-B14","doi-asserted-by":"publisher","first-page":"3542","DOI":"10.4049\/jimmunol.1403068","article-title":"Is the complement activation product C3a a proinflammatory molecule? Re-evaluating the evidence and the myth","volume":"194","author":"Coulthard","year":"2015","journal-title":"J Immunol"},{"key":"2025093003514440300_vkaf065-B15","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1111\/imr.12461","article-title":"Terminal complexes of the complement system: new structural insights and their relevance to function","volume":"274","author":"Morgan","year":"2016","journal-title":"Immunol Rev"},{"key":"2025093003514440300_vkaf065-B16","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1016\/s0161-5890(00)00024-9","article-title":"Complement factor: sequence analysis of 221 kb of human genomic DNA containing the entire fH, fHR-1 and fHR-3 genes","volume":"37","author":"Male","year":"2000","journal-title":"Mol Immunol"},{"key":"2025093003514440300_vkaf065-B17","doi-asserted-by":"publisher","first-page":"380","DOI":"10.1016\/j.it.2008.04.008","article-title":"Factor family proteins and human diseases","volume":"29","author":"J\u00f3zsi","year":"2008","journal-title":"Trends Immunol"},{"key":"2025093003514440300_vkaf065-B18","doi-asserted-by":"publisher","first-page":"E4433","DOI":"10.1073\/pnas.1717600115","article-title":"Recurrent structural variation, clustered sites of selection, and disease risk for the complement factor (CFH) gene family","volume":"115","author":"Cantsilieris","year":"2018","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2025093003514440300_vkaf065-B19","doi-asserted-by":"publisher","first-page":"415","DOI":"10.1053\/j.ajkd.2004.10.018","article-title":"Successful plasma therapy for atypical hemolytic uremic syndrome caused by factor deficiency owing to a novel mutation in the complement cofactor protein domain 15","volume":"45","author":"Licht","year":"2005","journal-title":"Am J Kidney Dis"},{"key":"2025093003514440300_vkaf065-B20","doi-asserted-by":"publisher","first-page":"747","DOI":"10.1016\/j.kint.2021.07.023","article-title":"Complement factor: a guardian within?","volume":"100","author":"Boudhabhay","year":"2021","journal-title":"Kidney Int"},{"key":"2025093003514440300_vkaf065-B21","doi-asserted-by":"publisher","first-page":"1728","DOI":"10.3389\/fimmu.2020.01728","article-title":"Characterization of binding properties of individual functional sites of human complement factor","volume":"11","author":"Haque","year":"2020","journal-title":"Front Immunol"},{"key":"2025093003514440300_vkaf065-B22","doi-asserted-by":"publisher","first-page":"728","DOI":"10.1038\/ni.1755","article-title":"Structure of complement fragment C3b-factor and implications for host protection by complement regulators","volume":"10","author":"Wu","year":"2009","journal-title":"Nat Immunol"},{"issue":"Pt 2","key":"2025093003514440300_vkaf065-B23","doi-asserted-by":"publisher","first-page":"321","DOI":"10.1042\/bj3260321","article-title":"The human complement regulatory factor-H-like protein 1, which represents a truncated form of factor, displays cell-attachment activity","volume":"326","author":"Hellwage","year":"1997","journal-title":"Biochem J"},{"key":"2025093003514440300_vkaf065-B24","doi-asserted-by":"publisher","first-page":"4198","DOI":"10.1096\/fj.11-186460","article-title":"Monomeric C-reactive protein modulates classic complement activation on necrotic cells","volume":"25","author":"Mihlan","year":"2011","journal-title":"Faseb J"},{"key":"2025093003514440300_vkaf065-B25","doi-asserted-by":"publisher","first-page":"76","DOI":"10.1038\/nature10449","article-title":"Complement factor binds malondialdehyde epitopes and protects from oxidative stress","volume":"478","author":"Weismann","year":"2011","journal-title":"Nature"},{"key":"2025093003514440300_vkaf065-B26","doi-asserted-by":"publisher","first-page":"27657","DOI":"10.1074\/jbc.M002903200","article-title":"Each of the three binding sites on complement factor interacts with a distinct site on C3b","volume":"275","author":"Jokiranta","year":"2000","journal-title":"J Biol Chem"},{"key":"2025093003514440300_vkaf065-B27","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1016\/j.jmb.2009.10.010","article-title":"The central portion of factor (modules 10-15) is compact and contains a structurally deviant CCP module","volume":"395","author":"Schmidt","year":"2010","journal-title":"J Mol Biol"},{"key":"2025093003514440300_vkaf065-B28","doi-asserted-by":"publisher","first-page":"107624","DOI":"10.1016\/j.jbc.2024.107624","article-title":"The SCR-17 and SCR-18 glycans in human complement factor enhance its regulatory function","volume":"300","author":"Gao","year":"2024","journal-title":"J Biol Chem"},{"key":"2025093003514440300_vkaf065-B29","doi-asserted-by":"publisher","first-page":"463","DOI":"10.1038\/nsmb.2018","article-title":"Structural basis for engagement by complement factor of C3b on a self surface","volume":"18","author":"Morgan","year":"2011","journal-title":"Nat Struct Mol Biol"},{"key":"2025093003514440300_vkaf065-B30","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1159\/000019124","article-title":"Conservation of plasma regulatory proteins of the complement system in evolution: humans and fish","volume":"17","author":"Kemper","year":"2000","journal-title":"Exp Clin Immunogenet"},{"key":"2025093003514440300_vkaf065-B31","doi-asserted-by":"publisher","first-page":"217","DOI":"10.1084\/jem.111.2.217","article-title":"Relation of a beta(1)-glycoprotein of human serum to the complement system","volume":"111","author":"M\u00fcller-Eberhard","year":"1960","journal-title":"J Exp Med"},{"key":"2025093003514440300_vkaf065-B32","doi-asserted-by":"publisher","first-page":"261","DOI":"10.1016\/j.immuni.2017.01.006","article-title":"Complement factor inhibits CD47-mediated resolution of inflammation","volume":"46","author":"Calippe","year":"2017","journal-title":"Immunity"},{"key":"2025093003514440300_vkaf065-B33","doi-asserted-by":"publisher","first-page":"122","DOI":"10.1158\/2326-6066.CIR-23-1092","article-title":"Complement factor is an ICOS ligand modulating tregs in the glioma microenvironment","volume":"13","author":"Smolag","year":"2025","journal-title":"Cancer Immunol Res"},{"key":"2025093003514440300_vkaf065-B34","doi-asserted-by":"publisher","first-page":"4538","DOI":"10.1182\/blood-2009-03-205096","article-title":"Platelet-associated complement factor in healthy persons and patients with atypical HUS","volume":"114","author":"Licht","year":"2009","journal-title":"Blood"},{"key":"2025093003514440300_vkaf065-B35","doi-asserted-by":"publisher","first-page":"44","DOI":"10.1002\/eji.202470300","article-title":"An enemy within: Intracellular complement factor fuels tumor progression via cell cycle acceleration and enhanced energy metabolism","volume":"54","author":"Artero","year":"2023","journal-title":"Eur J Immunol"},{"key":"2025093003514440300_vkaf065-B36","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/eji.202470300","article-title":"Roumenina, An enemy within: Intracellular complement factor fuels tumor progression via cell cycle acceleration and enhanced energy metabolism","volume":"54","author":"Artero","year":"2024","journal-title":"Euro J Immunol"},{"key":"2025093003514440300_vkaf065-B37","doi-asserted-by":"publisher","first-page":"903","DOI":"10.1038\/cdd.2015.164","article-title":"Factor H uptake regulates intracellular C3 activation during apoptosis and decreases the inflammatory potential of nucleosomes","volume":"23","author":"Martin","year":"2016","journal-title":"Cell Death Differ"},{"key":"2025093003514440300_vkaf065-B38","doi-asserted-by":"publisher","first-page":"1368852","DOI":"10.3389\/fimmu.2024.1368852","article-title":"Complement-coagulation cross-talk: factor-mediated regulation of the complement classical pathway activation by fibrin clots","volume":"15","author":"Kang","year":"2024","journal-title":"Front Immunol"},{"key":"2025093003514440300_vkaf065-B39","doi-asserted-by":"publisher","first-page":"809","DOI":"10.1016\/s0161-5890(99)00101-7","article-title":"FHL-1\/reconectin and factor: two human complement regulators which are encoded by the same gene are differently expressed and regulated","volume":"36","author":"Friese","year":"1999","journal-title":"Mol Immunol"},{"key":"2025093003514440300_vkaf065-B40","doi-asserted-by":"crossref","first-page":"2592","DOI":"10.4049\/jimmunol.149.8.2592","article-title":"Ultrastructures and interactions of complement factors H and I","volume":"149","author":"DiScipio","year":"1992","journal-title":"J Immunol"},{"key":"2025093003514440300_vkaf065-B41","doi-asserted-by":"crossref","first-page":"12015","DOI":"10.1016\/S0021-9258(18)99058-7","article-title":"Molecular cloning of a human serum protein structurally related to complement factor","volume":"266","author":"Skerka","year":"1991","journal-title":"J Biol Chem"},{"key":"2025093003514440300_vkaf065-B42","doi-asserted-by":"publisher","first-page":"4685","DOI":"10.1073\/pnas.1219260110","article-title":"Dimerization of complement factor-related proteins modulates complement activation in vivo","volume":"110","author":"Goicoechea de Jorge","year":"2013","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2025093003514440300_vkaf065-B43","doi-asserted-by":"publisher","first-page":"3484","DOI":"10.1182\/blood.2020010069","article-title":"Molecular bases for the association of FHR-1 with atypical hemolytic uremic syndrome and other diseases","volume":"137","author":"Merinero","year":"2021","journal-title":"Blood"},{"key":"2025093003514440300_vkaf065-B44","doi-asserted-by":"publisher","first-page":"615748","DOI":"10.3389\/fimmu.2021.615748","article-title":"Deregulation of factor by factor-related protein 1 depends on sialylation of host surfaces","volume":"12","author":"Dopler","year":"2021","journal-title":"Front Immunol"},{"key":"2025093003514440300_vkaf065-B45","doi-asserted-by":"crossref","first-page":"1469176","DOI":"10.3389\/frhem.2024.1469176","article-title":"Unique target binding by the C-terminal region of FHR1 provides a new perception of aHUS pathology","volume":"3","author":"Perie","year":"2024","journal-title":"Front Hematol"},{"key":"2025093003514440300_vkaf065-B46","doi-asserted-by":"publisher","first-page":"2961","DOI":"10.1038\/s41467-019-10766-0","article-title":"Serum FHR1 binding to necrotic-type cells activates monocytic inflammasome and marks necrotic sites in vasculopathies","volume":"10","author":"Irmscher","year":"2019","journal-title":"Nat Commun"},{"key":"2025093003514440300_vkaf065-B47","doi-asserted-by":"publisher","first-page":"1173","DOI":"10.1038\/ng1890","article-title":"A common CFH haplotype, with deletion of CFHR1 and CFHR3, is associated with lower risk of age-related macular degeneration","volume":"38","author":"Hughes","year":"2006","journal-title":"Nat Genet"},{"key":"2025093003514440300_vkaf065-B48","doi-asserted-by":"publisher","first-page":"321","DOI":"10.1038\/ng.787","article-title":"Genome-wide association study identifies susceptibility loci for IgA nephropathy","volume":"43","author":"Gharavi","year":"2011","journal-title":"Nat Genet"},{"key":"2025093003514440300_vkaf065-B49","doi-asserted-by":"publisher","first-page":"e41","DOI":"10.1371\/journal.pgen.0030041","article-title":"Deletion of complement factor-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome","volume":"3","author":"Zipfel","year":"2007","journal-title":"PLoS Genet"},{"key":"2025093003514440300_vkaf065-B50","doi-asserted-by":"publisher","first-page":"e1002079","DOI":"10.1371\/journal.pgen.1002079","article-title":"Association of genetic variants in complement factor and factor-related genes with systemic lupus erythematosus susceptibility","volume":"7","author":"Zhao","year":"2011","journal-title":"PLoS Genet"},{"key":"2025093003514440300_vkaf065-B51","doi-asserted-by":"publisher","first-page":"772","DOI":"10.1038\/ng.640","article-title":"Genome-wide association study identifies variants in the CFH region associated with host susceptibility to meningococcal disease","volume":"42","author":"Davila","year":"2010","journal-title":"Nat Genet"},{"key":"2025093003514440300_vkaf065-B52","doi-asserted-by":"publisher","first-page":"2079","DOI":"10.1016\/j.cell.2024.03.038","article-title":"Exploring new perspectives in immunology","volume":"187","author":"Medzhitov","year":"2024","journal-title":"Cell"},{"key":"2025093003514440300_vkaf065-B53","doi-asserted-by":"publisher","first-page":"912","DOI":"10.1038\/s41577-024-01102-9","article-title":"The danger theory of immunity revisited","volume":"24","author":"Kroemer","year":"2024","journal-title":"Nat Rev Immunol"},{"key":"2025093003514440300_vkaf065-B54","doi-asserted-by":"publisher","first-page":"765","DOI":"10.1093\/glycob\/cwy061","article-title":"Biophysical analysis of sialic acid recognition by the complement regulator factor","volume":"28","author":"Schmidt","year":"2018","journal-title":"Glycobiology"},{"key":"2025093003514440300_vkaf065-B55","doi-asserted-by":"publisher","first-page":"80","DOI":"10.1016\/j.jmb.2007.09.026","article-title":"The regulatory SCR-1\/5 and cell surface-binding SCR-16\/20 fragments of factor reveal partially folded-back solution structures and different self-associative properties","volume":"375","author":"Okemefuna","year":"2008","journal-title":"J Mol Biol"},{"key":"2025093003514440300_vkaf065-B56","doi-asserted-by":"publisher","first-page":"824","DOI":"10.1016\/j.kint.2021.05.033","article-title":"Local complement factor protects kidney endothelial cell structure and function","volume":"100","author":"Mahajan","year":"2021","journal-title":"Kidney Int"},{"key":"2025093003514440300_vkaf065-B57","doi-asserted-by":"publisher","first-page":"251","DOI":"10.14670\/HH-19.251","article-title":"Attachment of the soluble complement regulator factor to cell and tissue surfaces: relevance for pathology","volume":"19","author":"J\u00f3zsi","year":"2004","journal-title":"Histol Histopathol"},{"key":"2025093003514440300_vkaf065-B58","doi-asserted-by":"publisher","first-page":"4374","DOI":"10.4049\/jimmunol.1002488","article-title":"Complement regulation at necrotic cell lesions is impaired by the age-related macular degeneration-associated factor-H His402 risk variant","volume":"187","author":"Lauer","year":"2011","journal-title":"J Immunol"},{"key":"2025093003514440300_vkaf065-B59","doi-asserted-by":"publisher","first-page":"369","DOI":"10.1016\/j.kint.2024.05.015","article-title":"The role of complement in kidney disease: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) controversies conference","volume":"106","author":"Vivarelli","year":"2024","journal-title":"Kidney Int"},{"key":"2025093003514440300_vkaf065-B60","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1038\/s41581-018-0107-2","article-title":"C3 glomerulopathy\u2014understanding a rare complement-driven renal disease","volume":"15","author":"Smith","year":"2019","journal-title":"Nat Rev Nephrol"},{"key":"2025093003514440300_vkaf065-B61","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.imlet.2009.07.005","article-title":"Complement and immune defense: from innate immunity to human diseases","volume":"126","author":"Zipfel","year":"2009","journal-title":"Immunol Lett"},{"key":"2025093003514440300_vkaf065-B62","doi-asserted-by":"publisher","first-page":"2418","DOI":"10.1002\/1873-3468.12284","article-title":"Self-nonself discrimination by the complement system","volume":"590","author":"Meri","year":"2016","journal-title":"FEBS Lett"},{"key":"2025093003514440300_vkaf065-B63","doi-asserted-by":"publisher","first-page":"241","DOI":"10.1681\/ASN.2019050515","article-title":"CFHR gene variations provide insights in the pathogenesis of the kidney diseases atypical hemolytic uremic syndrome and C3 glomerulopathy","volume":"31","author":"Zipfel","year":"2020","journal-title":"J Am Soc Nephrol"},{"key":"2025093003514440300_vkaf065-B64","doi-asserted-by":"publisher","first-page":"939","DOI":"10.1084\/jem.175.4.939","article-title":"Activation of the alternative pathway of complement by monoclonal lambda light chains in membranoproliferative glomerulonephritis","volume":"175","author":"Meri","year":"1992","journal-title":"J Exp Med"},{"key":"2025093003514440300_vkaf065-B65","doi-asserted-by":"publisher","first-page":"e1003308","DOI":"10.1371\/journal.ppat.1003308","article-title":"Microbes bind complement inhibitor factor via a common site","volume":"9","author":"Meri","year":"2013","journal-title":"PLoS Pathog"},{"key":"2025093003514440300_vkaf065-B66","doi-asserted-by":"publisher","first-page":"21","DOI":"10.1016\/j.molimm.2015.03.012","article-title":"The role of complement in C3 glomerulopathy","volume":"67","author":"Zipfel","year":"2015","journal-title":"Mol Immunol"},{"key":"2025093003514440300_vkaf065-B67","doi-asserted-by":"publisher","first-page":"607211","DOI":"10.3389\/fimmu.2020.607211","article-title":"Factor autoantibodies and complement-mediated diseases","volume":"11","author":"Zhang","year":"2020","journal-title":"Front Immunol"},{"key":"2025093003514440300_vkaf065-B68","doi-asserted-by":"publisher","first-page":"200","DOI":"10.1016\/j.molimm.2012.05.009","article-title":"Factor autoantibodies in membranoproliferative glomerulonephritis","volume":"52","author":"Goodship","year":"2012","journal-title":"Mol Immunol"},{"key":"2025093003514440300_vkaf065-B69","doi-asserted-by":"publisher","first-page":"582","DOI":"10.1136\/jmg.2005.038315","article-title":"Variations in the complement regulatory genes factor (CFH) and factor related 5 (CFHR5) are associated with membranoproliferative glomerulonephritis type II (dense deposit disease)","volume":"43","author":"Abrera-Abeleda","year":"2006","journal-title":"J Med Genet"},{"key":"2025093003514440300_vkaf065-B70","doi-asserted-by":"publisher","first-page":"1254759","DOI":"10.3389\/fimmu.2023.1254759","article-title":"Genetic investigation of Nordic patients with complement-mediated kidney diseases","volume":"14","author":"Rydberg","year":"2023","journal-title":"Front Immunol"},{"key":"2025093003514440300_vkaf065-B71","doi-asserted-by":"publisher","first-page":"2027","DOI":"10.1016\/S0002-9440(10)64481-1","article-title":"The molecular basis for hereditary porcine membranoproliferative glomerulonephritis type II: point mutations in the factor coding sequence block protein secretion","volume":"161","author":"Hegasy","year":"2002","journal-title":"Am J Pathol"},{"key":"2025093003514440300_vkaf065-B72","doi-asserted-by":"publisher","first-page":"424","DOI":"10.1038\/ng912","article-title":"Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor","volume":"31","author":"Pickering","year":"2002","journal-title":"Nat Genet"},{"key":"2025093003514440300_vkaf065-B73","doi-asserted-by":"publisher","first-page":"543","DOI":"10.1038\/s41581-021-00424-4","article-title":"Thrombotic microangiopathy in aHUS and beyond: clinical clues from complement genetics","volume":"17","author":"Fakhouri","year":"2021","journal-title":"Nat Rev Nephrol"},{"key":"2025093003514440300_vkaf065-B74","doi-asserted-by":"publisher","first-page":"1516","DOI":"10.1182\/blood-2007-02-071472","article-title":"Anti factor autoantibodies block C-terminal recognition function of factor in hemolytic uremic syndrome","volume":"110","author":"J\u00f3zsi","year":"2007","journal-title":"Blood"},{"key":"2025093003514440300_vkaf065-B75","doi-asserted-by":"publisher","first-page":"2180","DOI":"10.1681\/ASN.2010030315","article-title":"Clinical features of anti-factor autoantibody-associated hemolytic uremic syndrome","volume":"21","author":"Dragon-Durey","year":"2010","journal-title":"J Am Soc Nephrol"},{"key":"2025093003514440300_vkaf065-B76","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1038\/s41572-021-00265-2","article-title":"Age-related macular degeneration","volume":"7","author":"Fleckenstein","year":"2021","journal-title":"Nat Rev Dis Primers"},{"key":"2025093003514440300_vkaf065-B77","doi-asserted-by":"publisher","first-page":"4487","DOI":"10.1007\/s00018-021-03796-9","article-title":"The complement system in age-related macular degeneration","volume":"78","author":"Armento","year":"2021","journal-title":"Cell Mol Life Sci"},{"key":"2025093003514440300_vkaf065-B78","doi-asserted-by":"publisher","first-page":"3398","DOI":"10.1016\/j.molimm.2007.02.012","article-title":"Defective complement control of factor (Y402H) and FHL-1 in age-related macular degeneration","volume":"44","author":"Skerka","year":"2007","journal-title":"Mol Immunol"},{"key":"2025093003514440300_vkaf065-B79","doi-asserted-by":"publisher","first-page":"14517","DOI":"10.1038\/srep14517","article-title":"Pharmacogenetics of complement factor Y402H polymorphism and treatment of neovascular AMD with anti-VEGF agents: a meta-analysis","volume":"5","author":"Chen","year":"2015","journal-title":"Sci Rep"},{"key":"2025093003514440300_vkaf065-B80","first-page":"4374","article-title":"Complement regulation at necrotic cell lesions is impaired by the age-related macular degeneration-associated factor-H His402 risk variant","volume-title":"J Immunol","author":"Lauer","year":"2015"},{"key":"2025093003514440300_vkaf065-B81","doi-asserted-by":"publisher","first-page":"1621","DOI":"10.3390\/biom11111621","article-title":"Complement factor loss in RPE cells causes retinal degeneration in a human RPE-porcine retinal explant co-culture model","volume":"11","author":"Armento","year":"2021","journal-title":"Biomolecules"},{"key":"2025093003514440300_vkaf065-B82","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1007\/978-3-031-27681-1_2","article-title":"The noncanonical role of complement factor in retinal pigment epithelium (RPE) cells and implications for age-related macular degeneration (AMD)","volume":"1415","author":"Armento","year":"2023","journal-title":"Adv Exp Med Biol"},{"key":"2025093003514440300_vkaf065-B83","doi-asserted-by":"publisher","first-page":"199","DOI":"10.1172\/JCI86418","article-title":"VEGF regulates local inhibitory complement proteins in the eye and kidney","volume":"127","author":"Keir","year":"2017","journal-title":"J Clin Invest"},{"key":"2025093003514440300_vkaf065-B84","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1038\/s41581-024-00885-3","article-title":"The pathogenesis of IgA nephropathy and implications for treatment","volume":"21","author":"Cheung","year":"2025","journal-title":"Nat Rev Nephrol"},{"key":"2025093003514440300_vkaf065-B85","doi-asserted-by":"publisher","first-page":"109234","DOI":"10.1016\/j.intimp.2022.109234","article-title":"Complement factor variants are associated with microangiopathy lesions in IgA nephropathy","volume":"112","author":"Hou","year":"2022","journal-title":"Int Immunopharmacol"},{"key":"2025093003514440300_vkaf065-B86","doi-asserted-by":"publisher","first-page":"40","DOI":"10.1111\/nep.13580","article-title":"Variation in complement factor affects complement activation in immunoglobulin A vasculitis with nephritis","volume":"25","author":"Jia","year":"2020","journal-title":"Nephrology (Carlton)"},{"key":"2025093003514440300_vkaf065-B87","doi-asserted-by":"publisher","first-page":"1091","DOI":"10.1038\/s41588-023-01422-x","article-title":"Genome-wide association analyses define pathogenic signaling pathways and prioritize drug targets for IgA nephropathy","volume":"55","author":"Kiryluk","year":"2023","journal-title":"Nat Genet"},{"key":"2025093003514440300_vkaf065-B88","doi-asserted-by":"publisher","first-page":"953","DOI":"10.1016\/j.kint.2017.03.041","article-title":"Elevated factor-related protein 1 and factor pathogenic variants decrease complement regulation in IgA nephropathy","volume":"92","author":"Tortajada","year":"2017","journal-title":"Kidney Int"},{"key":"2025093003514440300_vkaf065-B89","doi-asserted-by":"publisher","first-page":"942","DOI":"10.1016\/j.kint.2017.03.043","article-title":"Circulating complement factor-related proteins 1 and 5 correlate with disease activity in IgA nephropathy","volume":"92","author":"Medjeral-Thomas","year":"2017","journal-title":"Kidney Int"},{"key":"2025093003514440300_vkaf065-B90","doi-asserted-by":"publisher","first-page":"22511","DOI":"10.1038\/s41598-021-02011-w","article-title":"Factor H-related protein 1 (FHR-1) is associated with atherosclerotic cardiovascular disease","volume":"11","author":"Irmscher","year":"2021","journal-title":"Sci Rep"},{"key":"2025093003514440300_vkaf065-B91","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1681\/ASN.0000000000000274","article-title":"The improved kidney risk score in ANCA-associated vasculitis for clinical practice and trials","volume":"35","author":"Bate","year":"2024","journal-title":"J Am Soc Nephrol"},{"key":"2025093003514440300_vkaf065-B92","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1056\/NEJMoa2023386","article-title":"Avacopan for the treatment of ANCA-associated vasculitis","volume":"384","author":"Jayne","year":"2021","journal-title":"N Engl J Med"},{"key":"2025093003514440300_vkaf065-B93","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1016\/j.clim.2016.11.013","article-title":"The functional activities of complement factor are impaired in patients with ANCA-positive vasculitis","volume":"175","author":"Chen","year":"2017","journal-title":"Clin Immunol"},{"key":"2025093003514440300_vkaf065-B94","doi-asserted-by":"publisher","first-page":"559","DOI":"10.3389\/fimmu.2018.00559","article-title":"Complement factor inhibits anti-neutrophil cytoplasmic autoantibody-induced neutrophil activation by interacting with neutrophils","volume":"9","author":"Chen","year":"2018","journal-title":"Front Immunol"},{"key":"2025093003514440300_vkaf065-B95","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1016\/j.kint.2023.10.013","article-title":"Complement alternative pathway determines disease susceptibility and severity in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis","volume":"105","author":"Lucientes-Continente","year":"2024","journal-title":"Kidney Int"},{"key":"2025093003514440300_vkaf065-B96","doi-asserted-by":"publisher","first-page":"2213","DOI":"10.1093\/rheumatology\/kex529","article-title":"Myeloperoxidase influences the complement regulatory activity of complement factor","volume":"57","author":"Chen","year":"2018","journal-title":"Rheumatology (Oxford)"},{"key":"2025093003514440300_vkaf065-B97","doi-asserted-by":"publisher","first-page":"698","DOI":"10.1038\/s41568-019-0210-0","article-title":"Context-dependent roles of complement in cancer","volume":"19","author":"Roumenina","year":"2019","journal-title":"Nat Rev Cancer"},{"key":"2025093003514440300_vkaf065-B98","doi-asserted-by":"publisher","first-page":"1578","DOI":"10.1158\/2326-6066.CIR-23-0399","article-title":"The yin yang of complement and cancer","volume":"11","author":"Meri","year":"2023","journal-title":"Cancer Immunol Res"},{"key":"2025093003514440300_vkaf065-B99","doi-asserted-by":"publisher","first-page":"6075","DOI":"10.4049\/jimmunol.164.11.6075","article-title":"Exceptional resistance of human H2 glioblastoma cells to complement-mediated killing by expression and utilization of factor and factor-like protein 1","volume":"164","author":"Junnikkala","year":"2000","journal-title":"J Immunol"},{"key":"2025093003514440300_vkaf065-B100","doi-asserted-by":"publisher","first-page":"909","DOI":"10.1158\/2326-6066.CIR-20-0787","article-title":"Intracellular factor drives tumor progression independently of the complement cascade","volume":"9","author":"Daugan","year":"2021","journal-title":"Cancer Immunol Res"},{"key":"2025093003514440300_vkaf065-B101","doi-asserted-by":"publisher","first-page":"1731135","DOI":"10.1080\/2162402X.2020.1731135","article-title":"Complement inhibitor factor expressed by breast cancer cells differentiates CD14+ human monocytes into immunosuppressive macrophages","volume":"9","author":"Smolag","year":"2020","journal-title":"Oncoimmunology"},{"key":"2025093003514440300_vkaf065-B102","doi-asserted-by":"publisher","first-page":"4039","DOI":"10.1172\/JCI135105","article-title":"Complement factor-deficient mice develop spontaneous hepatic tumors","volume":"130","author":"Laskowski","year":"2020","journal-title":"J Clin Invest"},{"key":"2025093003514440300_vkaf065-B103","doi-asserted-by":"publisher","first-page":"3226","DOI":"10.1158\/1078-0432.CCR-10-0321","article-title":"Complement factor autoantibodies are associated with early stage NSCLC","volume":"16","author":"Amornsiripanitch","year":"2010","journal-title":"Clin Cancer Res"},{"key":"2025093003514440300_vkaf065-B104","doi-asserted-by":"publisher","first-page":"5858","DOI":"10.1167\/iovs.09-5124","article-title":"Complement factor autoantibodies and age-related macular degeneration","volume":"51","author":"Dhillon","year":"2010","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"2025093003514440300_vkaf065-B105","doi-asserted-by":"publisher","first-page":"1589","DOI":"10.4049\/jimmunol.2300728","article-title":"Promotion of an antitumor immune program by a tumor-specific, complement-activating antibody","volume":"212","author":"Saxena","year":"2024","journal-title":"J Immunol"},{"key":"2025093003514440300_vkaf065-B106","doi-asserted-by":"publisher","first-page":"385","DOI":"10.3233\/CBM-210355","article-title":"Prognostic significance of a complement factor autoantibody in early stage NSCLC","volume":"34","author":"Gottlin","year":"2022","journal-title":"Cancer Biomark"},{"key":"2025093003514440300_vkaf065-B107","doi-asserted-by":"publisher","first-page":"375","DOI":"10.1016\/j.medcli.2024.05.019","volume-title":"Med Clin (Barc).","author":"Mihaylova","year":"2024"},{"key":"2025093003514440300_vkaf065-B108","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1007\/s13730-021-00627-5","article-title":"Thrombotic microangiopathy in a patient with systemic lupus erythematosus and anti-factor autoantibodies","volume":"11","author":"Figueiredo","year":"2022","journal-title":"CEN Case Rep"},{"key":"2025093003514440300_vkaf065-B109","first-page":"11","article-title":"Impact of autoantibodies to complement components on the disease activity in SLE","volume":"69","author":"Pradhan","year":"2021","journal-title":"J Assoc Physicians India"},{"key":"2025093003514440300_vkaf065-B110","doi-asserted-by":"publisher","first-page":"885","DOI":"10.3389\/fimmu.2019.00885","article-title":"Opposite profiles of complement in antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) among patients with antiphospholipid antibodies (aPL)","volume":"10","author":"Savelli","year":"2019","journal-title":"Front Immunol"},{"key":"2025093003514440300_vkaf065-B111","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1016\/j.thromres.2018.02.142","article-title":"Alternative pathway activation due to low level of complement factor in primary antiphospholipid syndrome","volume":"164","author":"Nakamura","year":"2018","journal-title":"Thromb Res"},{"key":"2025093003514440300_vkaf065-B112","doi-asserted-by":"publisher","first-page":"1786","DOI":"10.3899\/jrheum.150185","article-title":"Factor autoantibodies in patients with antiphospholipid syndrome and thrombosis","volume":"42","author":"Foltyn Zadura","year":"2015","journal-title":"J Rheumatol"},{"key":"2025093003514440300_vkaf065-B113","doi-asserted-by":"publisher","first-page":"R185","DOI":"10.1186\/ar4016","article-title":"Factor autoantibodies and deletion of complement factor-related protein-1 in rheumatic diseases in comparison to atypical hemolytic uremic syndrome","volume":"14","author":"Foltyn Zadura","year":"2012","journal-title":"Arthritis Res Ther"},{"key":"2025093003514440300_vkaf065-B114","doi-asserted-by":"publisher","first-page":"1089","DOI":"10.3109\/00365529109003961","article-title":"Occurrence of autoantibodies against intrinsic factor, H, K-ATPase, and pepsinogen in atrophic gastritis and rheumatoid arthritis","volume":"26","author":"M\u00e5rdh","year":"1991","journal-title":"Scand J Gastroenterol"},{"key":"2025093003514440300_vkaf065-B115","doi-asserted-by":"publisher","first-page":"2003","DOI":"10.1182\/blood-2013-05-501445","article-title":"Abnormalities in the alternative pathway of complement in children with hematopoietic stem cell transplant-associated thrombotic microangiopathy","volume":"122","author":"Jodele","year":"2013","journal-title":"Blood"},{"key":"2025093003514440300_vkaf065-B116","doi-asserted-by":"publisher","first-page":"2479","DOI":"10.1056\/NEJMc1805857","article-title":"Factor autoantibodies and membranous nephropathy","volume":"379","author":"Seikrit","year":"2018","journal-title":"N Engl J Med"},{"key":"2025093003514440300_vkaf065-B117","doi-asserted-by":"publisher","first-page":"1590","DOI":"10.1056\/NEJMc1905608","article-title":"More about factor autoantibodies in membranous nephropathy","volume":"381","author":"Valoti","year":"2019","journal-title":"N Engl J Med"},{"key":"2025093003514440300_vkaf065-B118","doi-asserted-by":"publisher","first-page":"82","DOI":"10.5414\/CN110479","article-title":"Association between anti-complement factor antibodies and renal outcome in primary membranous nephropathy","volume":"96","author":"Kagaya","year":"2021","journal-title":"Clin Nephrol"},{"key":"2025093003514440300_vkaf065-B119","doi-asserted-by":"publisher","first-page":"E98","DOI":"10.1002\/ajh.26868","article-title":"Factor H autoantibodies contribute to complement dysregulation in multisystem inflammatory syndrome in children (MIS-C)","volume":"98","author":"Zerra","year":"2023","journal-title":"Am J Hematol"},{"key":"2025093003514440300_vkaf065-B120","doi-asserted-by":"publisher","first-page":"660382","DOI":"10.3389\/fimmu.2021.660382","article-title":"Autoantibodies against the complement regulator factor in the serum of patients with neuromyelitis optica spectrum disorder","volume":"12","author":"Uzonyi","year":"2021","journal-title":"Front Immunol"},{"key":"2025093003514440300_vkaf065-B121","doi-asserted-by":"publisher","first-page":"842451","DOI":"10.3389\/fimmu.2022.842451","article-title":"Circulating levels of anti-C1q and anti-factor autoantibodies and their targets in normal pregnancy and preeclampsia","volume":"13","author":"Dijkstra","year":"2022","journal-title":"Front Immunol"},{"key":"2025093003514440300_vkaf065-B122","doi-asserted-by":"publisher","first-page":"387","DOI":"10.1042\/CS20230513","article-title":"The complement system in neurodegenerative diseases","volume":"138","author":"Tao","year":"2024","journal-title":"Clin Sci (Lond)"},{"key":"2025093003514440300_vkaf065-B123","doi-asserted-by":"publisher","first-page":"e12490","DOI":"10.1002\/dad2.12490","article-title":"Identification of an APOE \u03b54-specific blood-based molecular pathway for Alzheimer's disease risk","volume":"15","author":"Tao","year":"2023","journal-title":"Alzheimers Dement (Amst)"},{"key":"2025093003514440300_vkaf065-B124","doi-asserted-by":"publisher","first-page":"2586","DOI":"10.1002\/1873-3468.13758","article-title":"Complement evasion by the human respiratory tract pathogens Haemophilus influenzae and Moraxella catarrhalis","volume":"594","author":"Riesbeck","year":"2020","journal-title":"FEBS Lett"},{"key":"2025093003514440300_vkaf065-B125","doi-asserted-by":"publisher","first-page":"805","DOI":"10.1016\/j.tim.2023.02.011","article-title":"Meningococcal factor-binding protein: implications for disease susceptibility, virulence, and vaccines","volume":"31","author":"Yee","year":"2023","journal-title":"Trends Microbiol"},{"key":"2025093003514440300_vkaf065-B126","doi-asserted-by":"publisher","first-page":"594","DOI":"10.1093\/infdis\/jis718","article-title":"Glycerol-3-phosphate dehydrogenase 2 is a novel factor-, factor-like protein 1-, and plasminogen-binding surface protein of Candida albicans","volume":"207","author":"Luo","year":"2013","journal-title":"J Infect Dis"},{"key":"2025093003514440300_vkaf065-B127","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1016\/j.chom.2012.11.013","article-title":"Malaria parasites co-opt human factor to prevent complement-mediated lysis in the mosquito midgut","volume":"13","author":"Simon","year":"2013","journal-title":"Cell Host Microbe"},{"key":"2025093003514440300_vkaf065-B128","doi-asserted-by":"publisher","first-page":"e00393","DOI":"10.1128\/mSphere.00393-19","article-title":"Anti-factor antibody reactivity in young adults vaccinated with a meningococcal serogroup B vaccine containing factor binding protein","volume":"4","author":"Sharkey","year":"2019","journal-title":"mSphere"},{"key":"2025093003514440300_vkaf065-B129","doi-asserted-by":"publisher","first-page":"1809","DOI":"10.1016\/j.immuni.2023.06.026","article-title":"Cell-autonomous regulation of complement C3 by factor limits macrophage efferocytosis and exacerbates atherosclerosis","volume":"56","author":"Kiss","year":"2023","journal-title":"Immunity"}],"container-title":["The Journal of Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jimmunol\/article-pdf\/214\/9\/2150\/63159175\/vkaf065.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jimmunol\/article-pdf\/214\/9\/2150\/63159175\/vkaf065.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,30]],"date-time":"2025-09-30T07:51:58Z","timestamp":1759218718000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jimmunol\/article\/214\/9\/2150\/8129095"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,5,12]]},"references-count":129,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2025,5,12]]},"published-print":{"date-parts":[[2025,9,1]]}},"URL":"https:\/\/doi.org\/10.1093\/jimmun\/vkaf065","relation":{},"ISSN":["0022-1767","1550-6606"],"issn-type":[{"value":"0022-1767","type":"print"},{"value":"1550-6606","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2025,9]]},"published":{"date-parts":[[2025,5,12]]}}}