{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T10:41:23Z","timestamp":1790764883406,"version":"4.1.0"},"reference-count":47,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2019,11,8]],"date-time":"2019-11-08T00:00:00Z","timestamp":1573171200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2019,11,8]],"date-time":"2019-11-08T00:00:00Z","timestamp":1573171200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Research Fund Hungary","award":["PD116119"],"award-info":[{"award-number":["PD116119"]}]},{"name":"Bolyai J\u00e1nos Research Fellowship","award":["-"],"award-info":[{"award-number":["-"]}]},{"name":"Higher Education Institutional Excellence Program of the Ministry of Human Capacities in Hungary","award":["-"],"award-info":[{"award-number":["-"]}]},{"DOI":"10.13039\/501100002332","name":"Semmelweis University","doi-asserted-by":"crossref","award":["EFOP-3.6.3-VEKOP-16-2017-00009"],"award-info":[{"award-number":["EFOP-3.6.3-VEKOP-16-2017-00009"]}],"id":[{"id":"10.13039\/501100002332","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Orphanet J Rare Dis"],"published-print":{"date-parts":[[2019,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Acquired or genetic abnormalities of the complement alternative pathway are the primary cause of C3glomerulopathy(C3G) but may occur in immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) as well. Less is known about the presence and role of C4nephritic factor(C4NeF) which may stabilize the classical pathway C3-convertase. Our aim was to examine the presence of C4NeF and its connection with clinical features and with other pathogenic factors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>One hunfe IC-MPGN\/C3G patients were enrolled in the study. C4NeF activity was determined by hemolytic assay utilizing sensitized sheep erythrocytes. Seventeen patients were positive for C4NeF with lower prevalence of renal impairment and lower C4d level, and higher C3 nephritic factor (C3NeF) prevalence at time of diagnosis compared to C4NeF negative patients. Patients positive for both C3NeF and C4NeF had the lowest C3 levels and highest terminal pathway activation. End-stage renal disease did not develop in any of the C4NeF positive patients during follow-up period. Positivity to other complement autoantibodies (anti-C1q, anti-C3) was also linked to the presence of nephritic factors. Unsupervised, data-driven cluster analysis identified a group of patients with high prevalence of multiple complement autoantibodies, including C4NeF.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In conclusion, C4NeF may be a possible cause of complement dysregulation in approximately 10\u201315% of IC-MPGN\/C3G patients.<\/jats:p><\/jats:sec>","DOI":"10.1186\/s13023-019-1237-8","type":"journal-article","created":{"date-parts":[[2019,11,8]],"date-time":"2019-11-08T14:55:52Z","timestamp":1573224952000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy"],"prefix":"10.1186","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1959-4473","authenticated-orcid":false,"given":"N\u00f3ra","family":"Garam","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zolt\u00e1n","family":"Proh\u00e1szka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c1gnes","family":"Szil\u00e1gyi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christof","family":"Aigner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alice","family":"Schmidt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martina","family":"Gaggl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gere","family":"Sunder-Plassmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D\u00f3ra","family":"Bajcsi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00fcrgen","family":"Brunner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Dumfarth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Cejka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Flaschberger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hana","family":"Fl\u00f6gelova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c1gnes","family":"Haris","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c1gnes","family":"Hartmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Heilos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Mueller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Krisztina","family":"Rusai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus","family":"Arbeiter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes","family":"Hofer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D\u00e1niel","family":"Jakab","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e1ria","family":"Sink\u00f3","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erika","family":"Szigeti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Csaba","family":"Bereczki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Viktor","family":"Janko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kata","family":"Kelen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gy\u00f6rgy S.","family":"Reusz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Attila J.","family":"Szab\u00f3","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N\u00f3ra","family":"Klenk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Krisztina","family":"K\u00f3bor","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nika","family":"Kojc","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maarten","family":"Knechtelsdorfer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Laganovic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adrian Catalin","family":"Lungu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anamarija","family":"Meglic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rina","family":"Rus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tanja","family":"Kersnik-Levart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ernesta","family":"Macioniene","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marius","family":"Miglinas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Paw\u0142owska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomasz","family":"Stomp\u00f3r","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ludmila","family":"Podracka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Rudnicki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gert","family":"Mayer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Romana Rysava","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jana","family":"Reiterova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marijan","family":"Saraga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Tom\u00e1\u0161 Seeman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jakub","family":"Zieg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eva","family":"Sl\u00e1dkov\u00e1","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tam\u00e1s","family":"Szab\u00f3","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrei","family":"Capitanescu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simona","family":"Stancu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miroslav","family":"Tisljar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kresimir","family":"Galesic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andr\u00e1s","family":"Tisl\u00e9r","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inga","family":"Vainum\u00e4e","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Windpessl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomas","family":"Zaoral","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Galia","family":"Zlatanova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorottya","family":"Csuka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2019,11,8]]},"reference":[{"issue":"6","key":"1237_CR1","doi-asserted-by":"publisher","first-page":"479","DOI":"10.1016\/j.semnephrol.2013.08.001","volume":"33","author":"Marina Noris","year":"2013","unstructured":"Noris M, Remuzzi G. Overview of complement activation and regulation. Semin Nephrol 2013;33(6):479\u2013492. Epub 2013\/10\/29.","journal-title":"Seminars in Nephrology"},{"issue":"4","key":"1237_CR2","doi-asserted-by":"publisher","first-page":"454","DOI":"10.1038\/ki.2012.63","volume":"82","author":"A Servais","year":"2012","unstructured":"Servais A, Noel LH, Roumenina LT, Le Quintrec M, Ngo S, Dragon-Durey MA, et al. Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int. 2012;82(4):454\u201364 Epub 2012\/03\/30.","journal-title":"Kidney Int"},{"issue":"6","key":"1237_CR3","doi-asserted-by":"publisher","first-page":"1079","DOI":"10.1038\/ki.2013.377","volume":"84","author":"MC Pickering","year":"2013","unstructured":"Pickering MC, D'Agati VD, Nester CM, Smith RJ, Haas M, Appel GB, et al. C3 glomerulopathy: consensus report. Kidney Int. 2013;84(6):1079\u201389 Epub 2013\/11\/01.","journal-title":"Kidney Int"},{"issue":"5","key":"1237_CR4","doi-asserted-by":"publisher","first-page":"1603","DOI":"10.1681\/ASN.2016030343","volume":"28","author":"Maria Chiara Marinozzi","year":"2017","unstructured":"Marinozzi MC, Roumenina LT, Chauvet S, Hertig A, Bertrand D, Olagne J, et al. Anti-factor B and anti-C3b autoantibodies in C3 Glomerulopathy and Ig-associated Membranoproliferative GN. J Am Soc Nephrol 2017;28(5):1603\u20131613. Epub 2017\/01\/18.","journal-title":"Journal of the American Society of Nephrology"},{"key":"1237_CR5","doi-asserted-by":"publisher","first-page":"131","DOI":"10.1016\/j.molimm.2016.01.010","volume":"71","author":"Paraskevas Iatropoulos","year":"2016","unstructured":"Iatropoulos P, Noris M, Mele C, Piras R, Valoti E, Bresin E, et al. Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome. Mol Immunol 2016;71:131\u2013142. Epub 2016\/02\/20.","journal-title":"Molecular Immunology"},{"issue":"1","key":"1237_CR6","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1038\/sj.ki.5000269","volume":"70","author":"C. Licht","year":"2006","unstructured":"Licht C, Heinen S, Jozsi M, Loschmann I, Saunders RE, Perkins SJ, et al. Deletion of Lys224 in regulatory domain 4 of factor H reveals a novel pathomechanism for dense deposit disease (MPGN II). Kidney Int 2006;70(1):42\u201350. Epub 2006\/04\/14.","journal-title":"Kidney International"},{"issue":"8","key":"1237_CR7","doi-asserted-by":"publisher","first-page":"1551","DOI":"10.1681\/ASN.2010080795","volume":"22","author":"MA Abrera-Abeleda","year":"2011","unstructured":"Abrera-Abeleda MA, Nishimura C, Frees K, Jones M, Maga T, Katz LM, et al. Allelic variants of complement genes associated with dense deposit disease. J Am Soc Nephrol. 2011;22(8):1551\u20139 Epub 2011\/07\/26.","journal-title":"J Am Soc Nephrol"},{"issue":"11","key":"1237_CR8","doi-asserted-by":"publisher","first-page":"2425","DOI":"10.1681\/ASN.2013070732","volume":"25","author":"EK Wong","year":"2014","unstructured":"Wong EK, Anderson HE, Herbert AP, Challis RC, Brown P, Reis GS, et al. Characterization of a factor H mutation that perturbs the alternative pathway of complement in a family with membranoproliferative GN. J Am Soc Nephrol. 2014;25(11):2425\u201333 Epub 2014\/04\/12.","journal-title":"J Am Soc Nephrol"},{"issue":"6","key":"1237_CR9","doi-asserted-by":"publisher","first-page":"339","DOI":"10.4103\/0971-4065.134089","volume":"24","author":"GT John","year":"2014","unstructured":"Thomas S, Ranganathan D, Francis L, Madhan K, John GT. Current concepts in C3 glomerulopathy. Indian J Nephrol 2014;24(6):339\u2013348. Epub 2014\/12\/09.","journal-title":"Indian Journal of Nephrology"},{"issue":"1","key":"1237_CR10","doi-asserted-by":"publisher","first-page":"43","DOI":"10.1007\/s00467-015-3310-4","volume":"32","author":"M Riedl","year":"2017","unstructured":"Riedl M, Thorner P, Licht C. C3 Glomerulopathy. Pediatr Nephrol. 2017;32(1):43\u201357 Epub 2016\/04\/09.","journal-title":"Pediatr Nephrol"},{"key":"1237_CR11","doi-asserted-by":"publisher","first-page":"248","DOI":"10.12688\/f1000research.10364.1","volume":"6","author":"H. Terence Cook","year":"2017","unstructured":"Cook HT. C3 glomerulopathy. F1000Research. 2017;6:248. Epub 2017\/03\/31.","journal-title":"F1000Research"},{"issue":"6","key":"1237_CR12","doi-asserted-by":"publisher","first-page":"834","DOI":"10.1053\/j.ajkd.2017.07.004","volume":"70","author":"Y Zhang","year":"2017","unstructured":"Zhang Y, Meyer NC, Fervenza FC, Lau W, Keenan A, Cara-Fuentes G, et al. C4 nephritic factors in C3 Glomerulopathy: a case series. Am J Kidney Dis. 2017;70(6):834\u201343 Epub 2017\/08\/26.","journal-title":"Am J Kidney Dis"},{"issue":"5","key":"1237_CR13","doi-asserted-by":"publisher","first-page":"517","DOI":"10.1007\/s10875-016-0290-5","volume":"36","author":"AM Blom","year":"2016","unstructured":"Blom AM, Corvillo F, Magda M, Stasilojc G, Nozal P, Perez-Valdivia MA, et al. Testing the activity of complement Convertases in serum\/plasma for diagnosis of C4NeF-mediated C3 glomerulonephritis. J Clin Immunol. 2016;36(5):517\u201327 Epub 2016\/05\/06.","journal-title":"J Clin Immunol"},{"issue":"4","key":"1237_CR14","doi-asserted-by":"publisher","first-page":"465","DOI":"10.1038\/ki.2012.212","volume":"82","author":"S Sethi","year":"2012","unstructured":"Sethi S, Fervenza FC, Zhang Y, Zand L, Vrana JA, Nasr SH, et al. C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int. 2012;82(4):465\u201373 Epub 2012\/06\/08.","journal-title":"Kidney Int"},{"issue":"2","key":"1237_CR15","doi-asserted-by":"publisher","first-page":"265","DOI":"10.2215\/CJN.07900811","volume":"7","author":"Y Zhang","year":"2012","unstructured":"Zhang Y, Meyer NC, Wang K, Nishimura C, Frees K, Jones M, et al. Causes of alternative pathway dysregulation in dense deposit disease. Clin J Am Soc Nephrol. 2012;7(2):265\u201374 Epub 2012\/01\/10.","journal-title":"Clin J Am Soc Nephrol"},{"issue":"11","key":"1237_CR16","doi-asserted-by":"publisher","first-page":"5129","DOI":"10.4049\/jimmunol.1402770","volume":"194","author":"Caroline Blanc","year":"2015","unstructured":"Blanc C, Togarsimalemath SK, Chauvet S, Le Quintrec M, Moulin B, Buchler M, et al. Anti-factor H autoantibodies in C3 glomerulopathies and in atypical hemolytic uremic syndrome: one target, two diseases. J Immunol 2015;194(11):5129\u20135138. Epub 2015\/04\/29.","journal-title":"The Journal of Immunology"},{"issue":"2","key":"1237_CR17","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1038\/ki.2013.340","volume":"85","author":"Jean Hou","year":"2014","unstructured":"Hou J, Markowitz GS, Bomback AS, Appel GB, Herlitz LC, Barry Stokes M, et al. Toward a working definition of C3 glomerulopathy by immunofluorescence. Kidney Int 2014;85(2):450\u2013456. Epub 2013\/09\/27.","journal-title":"Kidney International"},{"issue":"11","key":"1237_CR18","doi-asserted-by":"publisher","first-page":"2326","DOI":"10.1016\/j.humpath.2014.07.021","volume":"45","author":"ML Figueres","year":"2014","unstructured":"Figueres ML, Fremeaux-Bacchi V, Rabant M, Galmiche L, Marinozzi MC, Grunfeld JP, et al. Heterogeneous histologic and clinical evolution in 3 cases of dense deposit disease with long-term follow-up. Hum Pathol. 2014;45(11):2326\u201333 Epub 2014\/09\/28.","journal-title":"Hum Pathol"},{"issue":"3878","key":"1237_CR19","doi-asserted-by":"publisher","first-page":"436","DOI":"10.1126\/science.164.3878.436","volume":"164","author":"R. E. Spitzer","year":"1969","unstructured":"Spitzer RE, Vallota EH, Forristal J, Sudora E, Stitzel A, Davis NC, et al. Serum C'3 lytic system in patients with glomerulonephritis. Science 1969;164(3878):436\u2013437. Epub 1969\/04\/25.","journal-title":"Science"},{"issue":"10","key":"1237_CR20","doi-asserted-by":"publisher","first-page":"1084","DOI":"10.1038\/ki.2012.250","volume":"82","author":"Danielle Paix\u00e3o-Cavalcante","year":"2012","unstructured":"Paixao-Cavalcante D, Lopez-Trascasa M, Skattum L, Giclas PC, Goodship TH, de Cordoba SR, et al. Sensitive and specific assays for C3 nephritic factors clarify mechanisms underlying complement dysregulation. Kidney Int 2012;82(10):1084\u20131092. Epub 2012\/08\/03.","journal-title":"Kidney International"},{"issue":"5","key":"1237_CR21","doi-asserted-by":"publisher","first-page":"1232","DOI":"10.1016\/j.kint.2017.04.017","volume":"92","author":"MC Marinozzi","year":"2017","unstructured":"Marinozzi MC, Chauvet S, Le Quintrec M, Mignotet M, Petitprez F, Legendre C, et al. C5 nephritic factors drive the biological phenotype of C3 glomerulopathies. Kidney Int. 2017;92(5):1232\u201341 Epub 2017\/07\/18.","journal-title":"Kidney Int"},{"issue":"6","key":"1237_CR22","doi-asserted-by":"publisher","first-page":"1249","DOI":"10.1172\/JCI109787","volume":"65","author":"L Halbwachs","year":"1980","unstructured":"Halbwachs L, Leveille M, Lesavre P, Wattel S, Leibowitch J. Nephritic factor of the classical pathway of complement: immunoglobulin G autoantibody directed against the classical pathway C3 convetase enzyme. J Clin Invest. 1980;65(6):1249\u201356 Epub 1980\/06\/01.","journal-title":"J Clin Invest"},{"issue":"2","key":"1237_CR23","doi-asserted-by":"publisher","first-page":"316","DOI":"10.1111\/j.1365-2249.1994.tb06530.x","volume":"95","author":"H Ohi","year":"1994","unstructured":"Ohi H, Yasugi T. Occurrence of C3 nephritic factor and C4 nephritic factor in membranoproliferative glomerulonephritis (MPGN). Clin Exp Immunol. 1994;95(2):316\u201321 Epub 1994\/02\/01.","journal-title":"Clin Exp Immunol"},{"issue":"1","key":"1237_CR24","first-page":"82","volume":"76","author":"Y Tanuma","year":"1989","unstructured":"Tanuma Y, Ohi H, Watanabe S, Seki M, Hatano M. C3 nephritic factor and C4 nephritic factor in the serum of two patients with hypocomplementaemic membranoproliferative glomerulonephritis. Clin Exp Immunol. 1989;76(1):82\u20135 Epub 1989\/04\/01.","journal-title":"Clin Exp Immunol"},{"key":"1237_CR25","doi-asserted-by":"crossref","unstructured":"Daha MR, van Es LA. Relative resistance of the F-42-stabilized classical pathway C3 convertase to inactivation by C4-binding protein. J Immunol 1980;125(5):2051\u20132054. Epub 1980\/11\/01.","DOI":"10.4049\/jimmunol.125.5.2051"},{"issue":"2","key":"1237_CR26","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1016\/j.imlet.2014.01.014","volume":"160","author":"M Jozsi","year":"2014","unstructured":"Jozsi M, Reuter S, Nozal P, Lopez-Trascasa M, Sanchez-Corral P, Prohaszka Z, et al. Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome. Immunol Lett. 2014;160(2):163\u201371 Epub 2014\/02\/05.","journal-title":"Immunol Lett"},{"issue":"7\u20138","key":"1237_CR27","doi-asserted-by":"publisher","first-page":"1476","DOI":"10.1016\/j.molimm.2010.02.002","volume":"47","author":"S Strobel","year":"2010","unstructured":"Strobel S, Zimmering M, Papp K, Prechl J, Jozsi M. Anti-factor B autoantibody in dense deposit disease. Mol Immunol. 2010;47(7\u20138):1476\u201383 Epub 2010\/03\/03.","journal-title":"Mol Immunol"},{"issue":"6","key":"1237_CR28","doi-asserted-by":"publisher","first-page":"455","DOI":"10.1111\/j.1365-2796.1997.tb00018.x","volume":"242","author":"L Skattum","year":"1997","unstructured":"Skattum L, Martensson U, Sjoholm AG. Hypocomplementaemia caused by C3 nephritic factors (C3 NeF): clinical findings and the coincidence of C3 NeF type II with anti-C1q autoantibodies. J Intern Med. 1997;242(6):455\u201364 Epub 1998\/01\/23.","journal-title":"J Intern Med"},{"issue":"1","key":"1237_CR29","doi-asserted-by":"publisher","first-page":"283","DOI":"10.1681\/ASN.2017030258","volume":"29","author":"P Iatropoulos","year":"2018","unstructured":"Iatropoulos P, Daina E, Curreri M, Piras R, Valoti E, Mele C, et al. Cluster analysis identifies distinct Pathogenetic patterns in C3 Glomerulopathies\/immune complex-mediated Membranoproliferative GN. J Am Soc Nephrol. 2018;29(1):283\u201394 Epub 2017\/10\/17.","journal-title":"J Am Soc Nephrol"},{"key":"1237_CR30","doi-asserted-by":"crossref","unstructured":"Garam N, Prohaszka Z, Rudniczki M, Aigner C, Lungu AC, Reiterova J, et al. Validation of pathogenic patterns in a novel cohort of patients with membranoproliferative glomerulonephritis by cluster analysis. Clin Kidney J. 2019.","DOI":"10.1016\/j.molimm.2018.06.074"},{"issue":"5","key":"1237_CR31","doi-asserted-by":"publisher","first-page":"1153","DOI":"10.1038\/ki.2015.227","volume":"88","author":"Cristina Rabasco","year":"2015","unstructured":"Rabasco C, Cavero T, Roman E, Rojas-Rivera J, Olea T, Espinosa M, et al. Effectiveness of mycophenolate mofetil in C3 glomerulonephritis. Kidney Int 2015;88(5):1153\u20131160. Epub 2015\/07\/30.","journal-title":"Kidney International"},{"issue":"4","key":"1237_CR32","doi-asserted-by":"publisher","first-page":"977","DOI":"10.1016\/j.kint.2017.10.022","volume":"93","author":"AS Bomback","year":"2018","unstructured":"Bomback AS, Santoriello D, Avasare RS, Regunathan-Shenk R, Canetta PA, Ahn W, et al. C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy. Kidney Int. 2018;93(4):977\u201385 Epub 2018\/01\/10.","journal-title":"Kidney Int"},{"key":"1237_CR33","unstructured":"Ito S, Tamura N, Fujita T. Effect of decay-accelerating factor on the assembly of the classical and alternative pathway C3 convertases in the presence of C4 or C3 nephritic factor. Immunology 1989;68(4):449\u2013452. Epub 1989\/12\/01."},{"issue":"3","key":"1237_CR34","doi-asserted-by":"publisher","first-page":"241","DOI":"10.1016\/j.clim.2012.09.007","volume":"145","author":"Elizabeth C. Miller","year":"2012","unstructured":"Miller EC, Chase NM, Densen P, Hintermeyer MK, Casper JT, Atkinson JP. Autoantibody stabilization of the classical pathway C3 convertase leading to C3 deficiency and Neisserial sepsis: C4 nephritic factor revisited. Clin Immunol 2012;145(3):241\u2013250. Epub 2012\/11\/03.","journal-title":"Clinical Immunology"},{"issue":"2","key":"1237_CR35","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1053\/j.ajkd.2015.03.040","volume":"66","author":"M Noris","year":"2015","unstructured":"Noris M, Remuzzi G. Glomerular diseases dependent on complement activation, including atypical hemolytic uremic syndrome, Membranoproliferative glomerulonephritis, and C3 Glomerulopathy: Core curriculum 2015. Am J Kidney Dis. 2015;66(2):359\u201375 Epub 2015\/06\/03.","journal-title":"Am J Kidney Dis"},{"key":"1237_CR36","doi-asserted-by":"crossref","unstructured":"Noris M, Donadelli R, Remuzzi G. Autoimmune abnormalities of the alternative complement pathway in membranoproliferative glomerulonephritis and C3 glomerulopathy. Pediatr Nephrol 2018. Epub 2018\/06\/28.","DOI":"10.1007\/s00467-018-3989-0"},{"key":"1237_CR37","doi-asserted-by":"publisher","first-page":"2329","DOI":"10.3389\/fimmu.2018.02329","volume":"9","author":"R Donadelli","year":"2018","unstructured":"Donadelli R, Pulieri P, Piras R, Iatropoulos P, Valoti E, Benigni A, et al. Unraveling the molecular mechanisms underlying complement Dysregulation by nephritic factors in C3G and IC-MPGN. Front Immunol. 2018;9:2329 Epub 2018\/11\/30.","journal-title":"Front Immunol"},{"issue":"4","key":"1237_CR38","first-page":"205","volume":"14","author":"TJ Fetterhoff","year":"1984","unstructured":"Fetterhoff TJ, McCarthy RC. A micromodification of the CH50 test for the classical pathway of complement. J Clin Lab Immunol. 1984;14(4):205\u20138 Epub 1984\/08\/01.","journal-title":"J Clin Lab Immunol"},{"issue":"5","key":"1237_CR39","doi-asserted-by":"publisher","first-page":"791","DOI":"10.1111\/j.1538-7836.2012.04674.x","volume":"10","author":"M Reti","year":"2012","unstructured":"Reti M, Farkas P, Csuka D, Razso K, Schlammadinger A, Udvardy ML, et al. Complement activation in thrombotic thrombocytopenic purpura. J Thromb Haemost. 2012;10(5):791\u20138 Epub 2012\/03\/01.","journal-title":"J Thromb Haemost"},{"issue":"1-2","key":"1237_CR40","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1016\/0022-1759(88)90160-3","volume":"114","author":"Christian Delamarche","year":"1988","unstructured":"Delamarche C, Berger F, Pouplard A, Emile J. An ELISA technique for the measurement of C1q in cerebrospinal fluid. J Immunol Methods 1988;114(1\u20132):101\u2013106. Epub 1988\/11\/10.","journal-title":"Journal of Immunological Methods"},{"issue":"2","key":"1237_CR41","doi-asserted-by":"publisher","first-page":"555","DOI":"10.1681\/ASN.2004050380","volume":"16","author":"MA Dragon-Durey","year":"2005","unstructured":"Dragon-Durey MA, Loirat C, Cloarec S, Macher MA, Blouin J, Nivet H, et al. Anti-factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol. 2005;16(2):555\u201363 Epub 2004\/12\/14.","journal-title":"J Am Soc Nephrol"},{"issue":"1","key":"1237_CR42","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1016\/0022-1759(82)90386-6","volume":"51","author":"U Rother","year":"1982","unstructured":"Rother U. A new screening test for C3 nephritis factor based on a stable cell bound convertase on sheep erythrocytes. J Immunol Methods. 1982;51(1):101\u20137 Epub 1982\/01\/01.","journal-title":"J Immunol Methods"},{"issue":"2","key":"1237_CR43","doi-asserted-by":"publisher","first-page":"367","DOI":"10.1016\/j.molimm.2015.02.013","volume":"65","author":"FC Mohlin","year":"2015","unstructured":"Mohlin FC, Nilsson SC, Levart TK, Golubovic E, Rusai K, Muller-Sacherer T, et al. Functional characterization of two novel non-synonymous alterations in CD46 and a Q950H change in factor H found in atypical hemolytic uremic syndrome patients. Mol Immunol. 2015;65(2):367\u201376 Epub 2015\/03\/04.","journal-title":"Mol Immunol"},{"issue":"1","key":"1237_CR44","doi-asserted-by":"publisher","first-page":"172","DOI":"10.1002\/eji.200939280","volume":"40","author":"Sara C. Nilsson","year":"2009","unstructured":"Nilsson SC, Kalchishkova N, Trouw LA, Fremeaux-Bacchi V, Villoutreix BO, Blom AM. Mutations in complement factor I as found in atypical hemolytic uremic syndrome lead to either altered secretion or altered function of factor I. Eur J Immunol 2010;40(1):172\u2013185. Epub 2009\/10\/31.","journal-title":"European Journal of Immunology"},{"issue":"4","key":"1237_CR45","doi-asserted-by":"publisher","first-page":"1267","DOI":"10.1182\/blood-2005-10-007252","volume":"108","author":"Jessica Caprioli","year":"2006","unstructured":"Caprioli J, Noris M, Brioschi S, Pianetti G, Castelletti F, Bettinaglio P, et al. Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood. 2006;108(4):1267\u20131279. Epub 2006\/04\/20.","journal-title":"Blood"},{"issue":"1\u20133","key":"1237_CR46","doi-asserted-by":"publisher","first-page":"111","DOI":"10.1016\/j.molimm.2006.07.004","volume":"44","author":"A Richards","year":"2007","unstructured":"Richards A, Kathryn Liszewski M, Kavanagh D, Fang CJ, Moulton E, Fremeaux-Bacchi V, et al. Implications of the initial mutations in membrane cofactor protein (MCP; CD46) leading to atypical hemolytic uremic syndrome. Mol Immunol. 2007;44(1\u20133):111\u201322 Epub 2006\/08\/03.","journal-title":"Mol Immunol"},{"issue":"2","key":"1237_CR47","doi-asserted-by":"publisher","first-page":"624","DOI":"10.1182\/blood-2007-04-084533","volume":"111","author":"Celia J. Fang","year":"2008","unstructured":"Fang CJ, Fremeaux-Bacchi V, Liszewski MK, Pianetti G, Noris M, Goodship TH, et al. Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome. Blood 2008;111(2):624\u2013632. Epub 2007\/10\/05.","journal-title":"Blood"}],"container-title":["Orphanet Journal of Rare Diseases"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-019-1237-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s13023-019-1237-8\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-019-1237-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,22]],"date-time":"2023-09-22T18:22:59Z","timestamp":1695406979000},"score":1,"resource":{"primary":{"URL":"https:\/\/ojrd.biomedcentral.com\/articles\/10.1186\/s13023-019-1237-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,11,8]]},"references-count":47,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2019,12]]}},"alternative-id":["1237"],"URL":"https:\/\/doi.org\/10.1186\/s13023-019-1237-8","relation":{},"ISSN":["1750-1172"],"issn-type":[{"value":"1750-1172","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,11,8]]},"assertion":[{"value":"25 July 2019","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 October 2019","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 November 2019","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Study protocol was approved by the Hungarian Medical Research Council (approval\u2019s number: 55381\u20131\/2015\/EKU) and the Institutional Review Board of the Semmelweis University, Budapest. Written approvals, based on informed consent, for the diagnostic tests and genetic analysis were given by the patients or their parents in accordance with the Declaration of Helsinki.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests as defined by Nature Research, or other interests that might be perceived to influence the results and\/or discussion reported in this paper.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"247"}}