{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T13:17:02Z","timestamp":1791206222222,"version":"4.1.0"},"reference-count":39,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2015,10,1]],"date-time":"2015-10-01T00:00:00Z","timestamp":1443657600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/501100003329","name":"Ministerio de Econom\u00eda y Competitividad","doi-asserted-by":"publisher","award":["PI1200597"],"award-info":[{"award-number":["PI1200597"]}],"id":[{"id":"10.13039\/501100003329","id-type":"DOI","asserted-by":"publisher"}]},{"name":"SRdeC","award":["SAF2011-26583"],"award-info":[{"award-number":["SAF2011-26583"]}]},{"name":"MLT","award":["SAF2012-38636"],"award-info":[{"award-number":["SAF2012-38636"]}]},{"name":"Autonomous Region of Madrid","award":["S2010\/BMD-2316"],"award-info":[{"award-number":["S2010\/BMD-2316"]}]},{"name":"Spanish Society of Nephrology (SENEFRO)"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Immunology"],"published-print":{"date-parts":[[2015,10]]},"DOI":"10.1016\/j.molimm.2015.06.021","type":"journal-article","created":{"date-parts":[[2015,7,8]],"date-time":"2015-07-08T00:48:15Z","timestamp":1436316495000},"page":"276-286","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":58,"special_numbering":"PB","title":["Complement factor H, FHR-3 and FHR-1 variants associate in an extended haplotype conferring increased risk of atypical hemolytic uremic syndrome"],"prefix":"10.1016","volume":"67","author":[{"given":"Maria E.","family":"Bernab\u00e9u-Herrero","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel","family":"Jim\u00e9nez-Alc\u00e1zar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaouad","family":"Anter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheila","family":"Pinto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"S\u00e1nchez Chinchilla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sof\u00eda","family":"Garrido","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margarita","family":"L\u00f3pez-Trascasa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Santiago","family":"Rodr\u00edguez de C\u00f3rdoba","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4212-1233","authenticated-orcid":false,"given":"Pilar","family":"S\u00e1nchez-Corral","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.molimm.2015.06.021_bib0005","doi-asserted-by":"crossref","first-page":"4261","DOI":"10.1182\/blood-2009-05-223834","article-title":"Characterization of complement factor H-related (CFHR) proteins in plasma reveals novel genetic variations of CFHR1 associated with atypical hemolytic uremic syndrome","volume":"114","author":"Abarrategui-Garrido","year":"2009","journal-title":"Blood"},{"key":"10.1016\/j.molimm.2015.06.021_bib0010","doi-asserted-by":"crossref","first-page":"3528","DOI":"10.4049\/jimmunol.1200679","article-title":"Overall neutralization of complement factor H by autoantibodies in the acute phase of the autoimmune form of atypical hemolytic uremic syndrome","volume":"189","author":"Blanc","year":"2012","journal-title":"J. Immunol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0200","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1681\/ASN.2012090884","article-title":"Combined complement gene mutations in atypical hemolytic uremic syndrome influence clinical phenotype","volume":"24","author":"Bresin","year":"2013","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0020","article-title":"Competition between antagonistic complement factors for a single protein on N. meningitidis rules disease susceptibility","volume":"23","author":"Caesar","year":"2014","journal-title":"eLIFE"},{"key":"10.1016\/j.molimm.2015.06.021_bib0205","doi-asserted-by":"crossref","first-page":"3385","DOI":"10.1093\/hmg\/ddg363","article-title":"Complement factor H mutations and gene polymorphisms in haemolytic uraemic syndrome: the C-257T, the A2089G and the G2881T polymorphisms are strongly associated with the disease","volume":"12","author":"Caprioli","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.molimm.2015.06.021_bib0210","doi-asserted-by":"crossref","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":"10.1016\/j.molimm.2015.06.021_bib0035","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1056\/NEJMoa0810739","article-title":"Thrombomodulin mutations in atypical hemolytic-uremic syndrome","volume":"361","author":"Delvaeye","year":"2009","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.molimm.2015.06.021_bib0040","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1681\/ASN.2004050380","article-title":"Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome","volume":"16","author":"Dragon-Durey","year":"2005","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0045","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1136\/jmg.2008.064766","article-title":"The high frequency of complement factor H related CFHR1 gene deletion is restricted to specific subgroups of patients with atypical haemolytic uraemic syndrome","volume":"46","author":"Dragon-Durey","year":"2009","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.molimm.2015.06.021_bib0050","doi-asserted-by":"crossref","first-page":"2180","DOI":"10.1681\/ASN.2010030315","article-title":"Clinical features of anti-factor H autoantibody-associated hemolytic uremic syndrome","volume":"21","author":"Dragon-Durey","year":"2010","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0055","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1093\/hmg\/ddi066","article-title":"Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32","volume":"14","author":"Esparza-Gordillo","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.molimm.2015.06.021_bib0060","doi-asserted-by":"crossref","first-page":"e84","DOI":"10.1136\/jmg.2004.019083","article-title":"Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome","volume":"41","author":"Fremeaux-Bacchi","year":"2004","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.molimm.2015.06.021_bib0065","doi-asserted-by":"crossref","first-page":"554","DOI":"10.2215\/CJN.04760512","article-title":"Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults","volume":"8","author":"Fremeaux-Bacchi","year":"2013","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0070","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1073\/pnas.0603420103","article-title":"Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome","volume":"104","author":"Goicoechea de Jorge","year":"2007","journal-title":"Proc. Natl. Acad. Sci."},{"key":"10.1016\/j.molimm.2015.06.021_bib0075","doi-asserted-by":"crossref","first-page":"7227","DOI":"10.1073\/pnas.0501536102","article-title":"A common haplotype in the complement regulatory gene factor H (HF1\/CFH) predisposes individuals to age-related macular degeneration","volume":"102","author":"Hageman","year":"2005","journal-title":"Proc. Natl. Acad. Sci."},{"key":"10.1016\/j.molimm.2015.06.021_bib0080","doi-asserted-by":"crossref","first-page":"1512","DOI":"10.1182\/blood-2007-09-109876","article-title":"Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1\/CFHR3 deficiency","volume":"111","author":"J\u00f3zsi","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.molimm.2015.06.021_bib0085","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1016\/j.semnephrol.2013.08.003","article-title":"Atypical hemolytic uremic syndrome","volume":"33","author":"Kavanagh","year":"2013","journal-title":"Semin. Nephrol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0090","doi-asserted-by":"crossref","first-page":"2169","DOI":"10.1056\/NEJMoa1208981","article-title":"Terminal complement inhibitor Eculizumab in atypical hemolytic-uremic syndrome","volume":"368","author":"Legendre","year":"2013","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.molimm.2015.06.021_bib0095","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1038\/ng.2590","article-title":"Recessive mutations in DGKE cause atypical hemolytic-uremic syndrome","volume":"45","author":"Lemaire","year":"2013","journal-title":"Nat. Genet."},{"key":"10.1016\/j.molimm.2015.06.021_bib0100","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1186\/1750-1172-6-60","article-title":"Atypical hemolytic uremic syndrome","volume":"6","author":"Loirat","year":"2011","journal-title":"Orphanet J. Rare Dis."},{"key":"10.1016\/j.molimm.2015.06.021_bib0105","doi-asserted-by":"crossref","first-page":"501","DOI":"10.4049\/jimmunol.177.1.501","article-title":"The meningococcal vaccine candidate GNA1870 binds the complement regulatory protein factor H and enhances serum resistance","volume":"177","author":"Madico","year":"2006","journal-title":"J. Immunol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0110","doi-asserted-by":"crossref","first-page":"E1445","DOI":"10.1002\/humu.21256","article-title":"Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndrome","volume":"31","author":"Maga","year":"2010","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.molimm.2015.06.021_bib0215","doi-asserted-by":"crossref","first-page":"1087","DOI":"10.1167\/iovs.11-8709","article-title":"Relevance of complement factor H-related 1 (CFHR1) genotypes in age-related macular degeneration","volume":"53","author":"Mart\u00ednez-Barricarte","year":"2012","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.molimm.2015.06.021_bib0120","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1182\/blood-2009-05-221549","article-title":"Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome","volume":"115","author":"Moore","year":"2010","journal-title":"Blood"},{"key":"10.1016\/j.molimm.2015.06.021_bib0125","doi-asserted-by":"crossref","first-page":"1844","DOI":"10.2215\/CJN.02210310","article-title":"Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype","volume":"5","author":"Noris","year":"2010","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0130","doi-asserted-by":"crossref","first-page":"1676","DOI":"10.1056\/NEJMra0902814","article-title":"Atypical hemolytic-uremic syndrome","volume":"361","author":"Noris","year":"2009","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.molimm.2015.06.021_bib0135","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1086\/318201","article-title":"Clustering of missense mutations in the C-terminal region of factor H in atypical hemolytic uremic syndrome","volume":"68","author":"P\u00e9rez-Caballero","year":"2001","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.molimm.2015.06.021_bib0140","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1038\/ki.2013.377","article-title":"C3 glomerulopathy: consensus report","volume":"84","author":"Pickering","year":"2013","journal-title":"Kidney Int."},{"key":"10.1016\/j.molimm.2015.06.021_bib0145","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1084\/jem.167.2.664","article-title":"A physical map of the human regulator of complement activation gene cluster linking the complement genes CR1, CR2, DAF, and C4BP","volume":"167","author":"Rey-Campos","year":"1988","journal-title":"J. Exp. Med."},{"key":"10.1016\/j.molimm.2015.06.021_bib0150","doi-asserted-by":"crossref","first-page":"12966","DOI":"10.1073\/pnas.2135497100","article-title":"Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome","volume":"100","author":"Richards","year":"2003","journal-title":"Proc. Natl. Acad. Sci."},{"key":"10.1016\/j.molimm.2015.06.021_bib0155","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1055\/s-0034-1375296","article-title":"Genetics of atypical hemolytic uremic syndrome (aHUS)","volume":"40","author":"Rodr\u00edguez de C\u00f3rdoba","year":"2014","journal-title":"Semin. Thromb. Hemost."},{"key":"10.1016\/j.molimm.2015.06.021_bib0160","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1007\/s00467-013-2591-8","article-title":"Eculizumab long-term therapy for pediatric renal transplant in aHUS with CFH\/CFHR1 hybrid gene","volume":"29","author":"Rom\u00e1n-Ortiz","year":"2014","journal-title":"Pediatr. Nephrol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0165","doi-asserted-by":"crossref","first-page":"1611","DOI":"10.2215\/CJN.01640214","article-title":"Complement mutations in diacylglycerol kinase-\u025b-associated atypical hemolytic uremic syndrome","volume":"9","author":"S\u00e1nchez Chinchilla","year":"2014","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0170","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1111\/j.1365-2141.2010.08295.x","article-title":"Advances in understanding the aetiology of atypical Haemolytic Uraemic Syndrome","volume":"150","author":"S\u00e1nchez-Corral","year":"2010","journal-title":"Br. J. Haematol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0175","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1136\/jmedgenet-2014-102498","article-title":"Factors determining penetrance in familial atypical haemolytic uraemic syndrome","volume":"51","author":"Sansbury","year":"2014","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.molimm.2015.06.021_bib0180","doi-asserted-by":"crossref","first-page":"7566","DOI":"10.4049\/jimmunol.176.12.7566","article-title":"Functional significance of factor H binding to Neisseria meningitidis","volume":"176","author":"Schneider","year":"2006","journal-title":"J. Immunol."},{"key":"10.1016\/j.molimm.2015.06.021_bib0185","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1038\/ki.2011.152","article-title":"Factor H-related protein 1 neutralizes anti-factor H autoantibodies in autoimmune hemolytic uremic syndrome","volume":"80","author":"Strobel","year":"2011","journal-title":"Kidney Int."},{"key":"10.1016\/j.molimm.2015.06.021_bib0190","doi-asserted-by":"crossref","first-page":"2434","DOI":"10.1172\/JCI68280","article-title":"C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation","volume":"123","author":"Tortajada","year":"2013","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.molimm.2015.06.021_bib0195","doi-asserted-by":"crossref","first-page":"e41","DOI":"10.1371\/journal.pgen.0030041","article-title":"Deletion of complement factor H-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome","volume":"3","author":"Zipfel","year":"2007","journal-title":"PLoS Genet."}],"container-title":["Molecular Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0161589015004459?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0161589015004459?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,9,20]],"date-time":"2018-09-20T22:54:41Z","timestamp":1537484081000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0161589015004459"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,10]]},"references-count":39,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2015,10]]}},"alternative-id":["S0161589015004459"],"URL":"https:\/\/doi.org\/10.1016\/j.molimm.2015.06.021","relation":{},"ISSN":["0161-5890"],"issn-type":[{"value":"0161-5890","type":"print"}],"subject":[],"published":{"date-parts":[[2015,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Complement factor H, FHR-3 and FHR-1 variants associate in an extended haplotype conferring increased risk of atypical hemolytic uremic syndrome","name":"articletitle","label":"Article Title"},{"value":"Molecular Immunology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.molimm.2015.06.021","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2015 Elsevier Ltd. All rights reserved.","name":"copyright","label":"Copyright"}]}}