{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T17:04:05Z","timestamp":1793898245057,"version":"4.8.0"},"reference-count":250,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2023,5,1]],"date-time":"2023-05-01T00:00:00Z","timestamp":1682899200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,5,1]],"date-time":"2023-05-01T00:00:00Z","timestamp":1682899200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2022,12,14]],"date-time":"2022-12-14T00:00:00Z","timestamp":1670976000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Autoimmunity"],"published-print":{"date-parts":[[2023,5]]},"DOI":"10.1016\/j.jaut.2022.102979","type":"journal-article","created":{"date-parts":[[2022,12,17]],"date-time":"2022-12-17T23:48:28Z","timestamp":1671320908000},"page":"102979","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":186,"special_numbering":"C","title":["The complement system and human autoimmune diseases"],"prefix":"10.1016","volume":"137","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4546-4488","authenticated-orcid":false,"given":"Samantha L.","family":"Coss","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danlei","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gilbert T.","family":"Chua","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rabheh Abdul","family":"Aziz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert P.","family":"Hoffman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yee Ling","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stacy P.","family":"Ardoin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John P.","family":"Atkinson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chack-Yung","family":"Yu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jaut.2022.102979_bib1","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1007\/978-94-017-8881-6_3","article-title":"Evolution of the complement system","volume":"80","author":"Nonaka","year":"2014","journal-title":"Subcell. Biochem."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib2","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1038\/sj.emboj.7600533","article-title":"The ancient origin of the complement system","volume":"24","author":"Zhu","year":"2005","journal-title":"EMBO J."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib3","first-page":"161","article-title":"Complement polymorphism, the major histocompatibility complex and associated diseases: a speculation","volume":"1","author":"Porter","year":"1983","journal-title":"Mol. Biol. Med."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib4","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1556\/EuJMI.2.2012.2.2","article-title":"The complement system: history, pathways, cascade and inhibitors","volume":"2","author":"Nesargikar","year":"2012","journal-title":"Eur J Microbiol Immunol (Bp)"},{"issue":"14","key":"10.1016\/j.jaut.2022.102979_bib5","doi-asserted-by":"crossref","first-page":"1058","DOI":"10.1056\/NEJM200104053441406","article-title":"Complement. First of two parts","volume":"344","author":"Walport","year":"2001","journal-title":"N. Engl. J. Med."},{"issue":"15","key":"10.1016\/j.jaut.2022.102979_bib6","doi-asserted-by":"crossref","first-page":"1140","DOI":"10.1056\/NEJM200104123441506","article-title":"Complement. Second of two parts","volume":"344","author":"Walport","year":"2001","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.jaut.2022.102979_bib7","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1146\/annurev.bi.50.070181.002245","article-title":"The proteolytic activation systems of complement","volume":"50","author":"Reid","year":"1981","journal-title":"Annu. Rev. Biochem."},{"key":"10.1016\/j.jaut.2022.102979_bib8","author":"Peters","year":"2022","journal-title":"Physiology and pathology of the C3 amplification cycle: A retrospective. Immunol Rev"},{"key":"10.1016\/j.jaut.2022.102979_bib9","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1111\/j.1600-065X.1999.tb01399.x","article-title":"Genomics of the major histocompatibility complex: haplotypes, duplication, retroviruses and disease","volume":"167","author":"Dawkins","year":"1999","journal-title":"Immunol. Rev."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib10","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1038\/s41590-020-00842-9","article-title":"Complement inhibitor for therapy of CHAPLE","volume":"22","author":"Yu","year":"2021","journal-title":"Nat. Immunol."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib11","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1128\/CMR.00048-09","article-title":"Infections of people with complement deficiencies and patients who have undergone splenectomy","volume":"23","author":"Ram","year":"2010","journal-title":"Clin. Microbiol. Rev."},{"issue":"14","key":"10.1016\/j.jaut.2022.102979_bib12","doi-asserted-by":"crossref","first-page":"1643","DOI":"10.1016\/j.molimm.2011.05.001","article-title":"Complement deficiency states and associated infections","volume":"48","author":"Skattum","year":"2011","journal-title":"Mol. Immunol."},{"key":"10.1016\/j.jaut.2022.102979_bib13","doi-asserted-by":"crossref","first-page":"694928","DOI":"10.3389\/fimmu.2021.694928","article-title":"Complement C4, infections, and autoimmune diseases","volume":"12","author":"Wang","year":"2021","journal-title":"Front. Immunol."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib14","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1542\/peds.71.1.81","article-title":"Inherited deficiency of the third component of complement associated with recurrent pyogenic infections, circulating immune complexes, and vasculitis in a Dutch family","volume":"71","author":"Roord","year":"1983","journal-title":"Pediatrics"},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib15","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1016\/j.jiac.2021.10.020","article-title":"Extreme gonococcal susceptibility associated with acquired complement deficiency secondary to hypocomplementemic urticarial vasculitis and systemic lupus erythematosus","volume":"28","author":"Belfeki","year":"2022","journal-title":"J. Infect. Chemother."},{"key":"10.1016\/j.jaut.2022.102979_bib16","doi-asserted-by":"crossref","first-page":"2237","DOI":"10.3389\/fimmu.2018.02237","article-title":"Interpretation of serological complement biomarkers in disease","volume":"9","author":"Ekdahl","year":"2018","journal-title":"Front. Immunol."},{"key":"10.1016\/j.jaut.2022.102979_bib17","doi-asserted-by":"crossref","first-page":"1450","DOI":"10.3389\/fimmu.2020.01450","article-title":"Viral evasion of the complement system and its importance for vaccines and therapeutics","volume":"11","author":"Mellors","year":"2020","journal-title":"Front. Immunol."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib18","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1046\/j.1365-2567.1999.00781.x","article-title":"Human mannan-binding lectin inhibits the infection of influenza A virus without complement","volume":"97","author":"Kase","year":"1999","journal-title":"Immunology"},{"key":"10.1016\/j.jaut.2022.102979_bib19","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1186\/1471-2172-11-64","article-title":"Lack of the pattern recognition molecule mannose-binding lectin increases susceptibility to influenza A virus infection","volume":"11","author":"Chang","year":"2010","journal-title":"BMC Immunol."},{"issue":"9","key":"10.1016\/j.jaut.2022.102979_bib20","doi-asserted-by":"crossref","first-page":"1484","DOI":"10.1128\/CVI.00527-09","article-title":"Mannose-binding lectin genotypes and susceptibility to epstein-barr virus infection in infancy","volume":"17","author":"Friborg","year":"2010","journal-title":"Clin. Vaccine Immunol."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib21","doi-asserted-by":"crossref","first-page":"2048","DOI":"10.4049\/jimmunol.0902772","article-title":"Influenza infection leads to increased susceptibility to subsequent bacterial superinfection by impairing NK cell responses in the lung","volume":"184","author":"Small","year":"2010","journal-title":"J. Immunol."},{"issue":"3\u20134","key":"10.1016\/j.jaut.2022.102979_bib22","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1111\/j.1574-695X.1999.tb01389.x","article-title":"Respiratory viral infection predisposing for bacterial disease: a concise review","volume":"26","author":"Hament","year":"1999","journal-title":"FEMS Immunol. Med. Microbiol."},{"issue":"9","key":"10.1016\/j.jaut.2022.102979_bib23","doi-asserted-by":"crossref","DOI":"10.3390\/v13091725","article-title":"Viral and bacterial Co-infections in the lungs: dangerous liaisons","volume":"13","author":"Oliva","year":"2021","journal-title":"Viruses"},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib24","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1172\/JCI110764","article-title":"Primate erythrocyte-immune complex-clearing mechanism","volume":"71","author":"Cornacoff","year":"1983","journal-title":"J. Clin. Invest."},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib25","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1056\/NEJM198608213150805","article-title":"The role of complement and its receptor in the elimination of immune complexes","volume":"315","author":"Schifferli","year":"1986","journal-title":"N. Engl. J. Med."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib26","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1016\/j.coi.2012.08.004","article-title":"Complement, interferon and lupus","volume":"24","author":"Elkon","year":"2012","journal-title":"Curr. Opin. Immunol."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib27","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1002\/art.1780270312","article-title":"Studies on the kinetics of binding of complement-fixing dsDNA\/anti-dsDNA immune complexes to the red blood cells of normal individuals and patients with systemic lupus erythematosus","volume":"27","author":"Horgan","year":"1984","journal-title":"Arthritis Rheum."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib28","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1016\/j.autrev.2008.06.002","article-title":"Immune complex clearance by complement receptor type 1 in SLE","volume":"8","author":"Kavai","year":"2008","journal-title":"Autoimmun. Rev."},{"issue":"2\u20133","key":"10.1016\/j.jaut.2022.102979_bib29","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1159\/000467850","article-title":"Complement-mediated solubilization of immune complexes and their interaction with complement C3 receptors","volume":"2","author":"Petersen","year":"1985","journal-title":"Complement"},{"issue":"Suppl 2","key":"10.1016\/j.jaut.2022.102979_bib30","first-page":"3","article-title":"Effects of plasma-exchange on complement-mediated solubilization of circulating immune complexes in patients with systemic lupus erythematosus","volume":"8","author":"Schena","year":"1985","journal-title":"Int. J. Artif. Organs"},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib31","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1111\/j.1365-3083.2010.02386.x","article-title":"Primary immunodeficiency and autoimmunity: lessons from human diseases","volume":"71","author":"Arason","year":"2010","journal-title":"Scand. J. Immunol."},{"key":"10.1016\/j.jaut.2022.102979_bib32","doi-asserted-by":"crossref","first-page":"55","DOI":"10.3389\/fimmu.2016.00055","article-title":"Systemic lupus erythematosus and deficiencies of early components of the complement classical pathway","volume":"7","author":"Macedo","year":"2016","journal-title":"Front. Immunol."},{"key":"10.1016\/j.jaut.2022.102979_bib33","doi-asserted-by":"crossref","first-page":"36","DOI":"10.3389\/fimmu.2016.00036","article-title":"Early components of the complement classical activation pathway in human systemic autoimmune diseases","volume":"7","author":"Lintner","year":"2016","journal-title":"Front. Immunol."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib34","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1111\/j.0022-202X.2005.23673.x","article-title":"C1q: its functions within the innate and adaptive immune responses and its role in lupus autoimmunity","volume":"125","author":"Sontheimer","year":"2005","journal-title":"J. Invest. Dermatol."},{"key":"10.1016\/j.jaut.2022.102979_bib35","doi-asserted-by":"crossref","first-page":"185","DOI":"10.3389\/fimmu.2015.00185","article-title":"Early-onset autoimmune disease as a manifestation of primary immunodeficiency","volume":"6","author":"Carneiro-Sampaio","year":"2015","journal-title":"Front. Immunol."},{"issue":"9","key":"10.1016\/j.jaut.2022.102979_bib36","doi-asserted-by":"crossref","first-page":"1601","DOI":"10.1136\/annrheumdis-2014-205287","article-title":"The complement system in systemic lupus erythematosus: an update","volume":"73","author":"Leffler","year":"2014","journal-title":"Ann. Rheum. Dis."},{"issue":"9","key":"10.1016\/j.jaut.2022.102979_bib37","doi-asserted-by":"crossref","first-page":"1096","DOI":"10.1177\/0961203310373370","article-title":"Cutaneous manifestations of complement deficiencies","volume":"19","author":"Lipsker","year":"2010","journal-title":"Lupus"},{"issue":"12","key":"10.1016\/j.jaut.2022.102979_bib38","doi-asserted-by":"crossref","first-page":"3574","DOI":"10.1002\/art.27720","article-title":"Regulation of complement by cartilage oligomeric matrix protein allows for a novel molecular diagnostic principle in rheumatoid arthritis","volume":"62","author":"Happonen","year":"2010","journal-title":"Arthritis Rheum."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib39","doi-asserted-by":"crossref","first-page":"997","DOI":"10.1002\/1529-0131(200105)44:5<997::AID-ANR178>3.0.CO;2-C","article-title":"Complement activation in patients with rheumatoid arthritis mediated in part by C-reactive protein","volume":"44","author":"Molenaar","year":"2001","journal-title":"Arthritis Rheum."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib40","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1136\/ard.58.5.309","article-title":"Increased apoptotic peripheral blood neutrophils in systemic lupus erythematosus: relations with disease activity, antibodies to double stranded DNA, and neutropenia","volume":"58","author":"Courtney","year":"1999","journal-title":"Ann. Rheum. Dis."},{"issue":"10","key":"10.1016\/j.jaut.2022.102979_bib41","doi-asserted-by":"crossref","first-page":"2888","DOI":"10.1002\/art.11237","article-title":"Increased apoptotic neutrophils and macrophages and impaired macrophage phagocytic clearance of apoptotic neutrophils in systemic lupus erythematosus","volume":"48","author":"Ren","year":"2003","journal-title":"Arthritis Rheum."},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib42","doi-asserted-by":"crossref","first-page":"597","DOI":"10.3109\/08916934.2012.719953","article-title":"Apoptosis and NET formation in the pathogenesis of SLE","volume":"45","author":"Bouts","year":"2012","journal-title":"Autoimmunity"},{"key":"10.1016\/j.jaut.2022.102979_bib43","doi-asserted-by":"crossref","first-page":"380","DOI":"10.3389\/fimmu.2012.00380","article-title":"Proteins derived from neutrophil extracellular traps may serve as self-antigens and mediate organ damage in autoimmune diseases","volume":"3","author":"Knight","year":"2012","journal-title":"Front. Immunol."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib44","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1177\/0961203308089990","article-title":"Apoptosis in the pathogenesis of systemic lupus erythematosus","volume":"17","author":"Munoz","year":"2008","journal-title":"Lupus"},{"issue":"12","key":"10.1016\/j.jaut.2022.102979_bib45","doi-asserted-by":"crossref","first-page":"716","DOI":"10.1038\/nrrheum.2016.186","article-title":"New insights into the immunopathogenesis of systemic lupus erythematosus","volume":"12","author":"Tsokos","year":"2016","journal-title":"Nat. Rev. Rheumatol."},{"issue":"136","key":"10.1016\/j.jaut.2022.102979_bib46","first-page":"75","article-title":"Triggers of inflammatory myopathy: insights into pathogenesis","volume":"25","author":"Adler","year":"2018","journal-title":"Discov. Med."},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib47","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1093\/rheumatology\/33.8.707","article-title":"Expression of complement regulatory molecules and other surface markers on neutrophils from synovial fluid and blood of patients with rheumatoid arthritis","volume":"33","author":"Jones","year":"1994","journal-title":"Br. J. Rheumatol."},{"key":"10.1016\/j.jaut.2022.102979_bib48","doi-asserted-by":"crossref","first-page":"958392","DOI":"10.3389\/fimmu.2022.958392","article-title":"Anaphylatoxins spark the flame in early autoimmunity","volume":"13","author":"Schanzenbacher","year":"2022","journal-title":"Front. Immunol."},{"key":"10.1016\/j.jaut.2022.102979_bib49","doi-asserted-by":"crossref","first-page":"S4","DOI":"10.1007\/s10875-007-9167-y","article-title":"Primary immunodeficiencies unravel critical aspects of the pathophysiology of autoimmunity and of the genetics of autoimmune disease","volume":"1","author":"Coutinho","year":"2008","journal-title":"J. Clin. Immunol."},{"key":"10.1016\/j.jaut.2022.102979_bib50","doi-asserted-by":"crossref","DOI":"10.1177\/2050312119876146","article-title":"Rheumatoid arthritis and systemic lupus erythematosus: pathophysiological mechanisms related to innate immune system","volume":"7","author":"Pab\u00f3n-Porras","year":"2019","journal-title":"SAGE Open Med"},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib51","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1111\/j.1749-6632.2012.06783.x","article-title":"The role of complement in experimental autoimmune myasthenia gravis","volume":"1274","author":"Kusner","year":"2012","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"10.1016\/j.jaut.2022.102979_bib52","doi-asserted-by":"crossref","first-page":"S34","DOI":"10.1007\/s10875-008-9187-2","article-title":"Understanding systemic lupus erythematosus physiopathology in the light of primary immunodeficiencies","volume":"1","author":"Carneiro-Sampaio","year":"2008","journal-title":"J. Clin. Immunol."},{"key":"10.1016\/j.jaut.2022.102979_bib53","first-page":"120","article-title":"Role of complement and other innate immune mechanisms in the removal of apoptotic cells","volume":"9","author":"Ogden","year":"2006","journal-title":"Curr. Dir. Autoimmun."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib54","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1136\/ard.2005.035733","article-title":"Reduced uptake of apoptotic cells by macrophages in systemic lupus erythematosus: correlates with decreased serum levels of complement","volume":"65","author":"Bijl","year":"2006","journal-title":"Ann. Rheum. Dis."},{"issue":"10","key":"10.1016\/j.jaut.2022.102979_bib55","doi-asserted-by":"crossref","first-page":"6175","DOI":"10.4049\/jimmunol.0902232","article-title":"C1q differentially modulates phagocytosis and cytokine responses during ingestion of apoptotic cells by human monocytes, macrophages, and dendritic cells","volume":"183","author":"Fraser","year":"2009","journal-title":"J. Immunol."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib56","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1007\/s12016-019-08774-5","article-title":"The immunopathology of complement proteins and innate immunity in autoimmune disease","volume":"58","author":"Defendi","year":"2020","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"10.1016\/j.jaut.2022.102979_bib57","doi-asserted-by":"crossref","first-page":"102540","DOI":"10.1016\/j.jaut.2020.102540","article-title":"Extracellular vesicles and lupus nephritis - new insights into pathophysiology and clinical implications","volume":"115","author":"Zhao","year":"2020","journal-title":"J. Autoimmun."},{"key":"10.1016\/j.jaut.2022.102979_bib58","doi-asserted-by":"crossref","first-page":"402","DOI":"10.3389\/fimmu.2014.00402","article-title":"Complement, c1q, and c1q-related molecules regulate macrophage polarization","volume":"5","author":"Bohlson","year":"2014","journal-title":"Front. Immunol."},{"issue":"10","key":"10.1016\/j.jaut.2022.102979_bib59","doi-asserted-by":"crossref","first-page":"3081","DOI":"10.1002\/art.24852","article-title":"C1q inhibits immune complex-induced interferon-alpha production in plasmacytoid dendritic cells: a novel link between C1q deficiency and systemic lupus erythematosus pathogenesis","volume":"60","author":"Lood","year":"2009","journal-title":"Arthritis Rheum."},{"issue":"Suppl 3","key":"10.1016\/j.jaut.2022.102979_bib60","doi-asserted-by":"crossref","first-page":"S279","DOI":"10.1186\/ar586","article-title":"Complement and systemic lupus erythematosus","volume":"4","author":"Walport","year":"2002","journal-title":"Arthritis Res."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib61","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1016\/j.molimm.2014.06.030","article-title":"Are complement deficiencies really rare? Overview on prevalence, clinical importance and modern diagnostic approach","volume":"61","author":"Grumach","year":"2014","journal-title":"Mol. Immunol."},{"issue":"1\u20132","key":"10.1016\/j.jaut.2022.102979_bib62","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1016\/j.molimm.2005.06.025","article-title":"Complement deficiency and disease: an update","volume":"43","author":"Sjoholm","year":"2006","journal-title":"Mol. Immunol."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib63","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1097\/00005792-198409000-00001","article-title":"Complement deficiency states and infection: epidemiology, pathogenesis and consequences of neisserial and other infections in an immune deficiency","volume":"63","author":"Ross","year":"1984","journal-title":"Medicine (Baltim.)"},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib64","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1542\/pir.22-11-365","article-title":"Hemolytic-uremic syndrome","volume":"22","author":"Corrigan","year":"2001","journal-title":"Pediatr. Rev."},{"issue":"17","key":"10.1016\/j.jaut.2022.102979_bib65","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."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib66","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1182\/blood-2005-10-007252","article-title":"International Registry of R, Familial HT. Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome","volume":"108","author":"Caprioli","year":"2006","journal-title":"Blood"},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib67","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1681\/ASN.V102281","volume":"10","author":"Noris","year":"1999","journal-title":"J. Am. Soc. Nephrol."},{"key":"10.1016\/j.jaut.2022.102979_bib68","doi-asserted-by":"crossref","DOI":"10.1177\/2054358119897229","article-title":"Clinical characteristics and outcome of Canadian patients diagnosed with atypical hemolytic uremic syndrome","volume":"7","author":"Lapeyraque","year":"2020","journal-title":"Can J Kidney Health Dis"},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib69","doi-asserted-by":"crossref","first-page":"2677","DOI":"10.1002\/art.34473","article-title":"Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus","volume":"64","author":"Petri","year":"2012","journal-title":"Arthritis Rheum."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib70","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1038\/ki.1991.102","article-title":"Diagnostic significance of hypocomplementemia","volume":"39","author":"Hebert","year":"1991","journal-title":"Kidney Int."},{"issue":"7","key":"10.1016\/j.jaut.2022.102979_bib71","doi-asserted-by":"crossref","first-page":"793","DOI":"10.1002\/art.1780250715","article-title":"Complement and lupus erythematosus","volume":"25","author":"Schur","year":"1982","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.jaut.2022.102979_bib72","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1007\/0-387-34134-X_16","article-title":"Three distinct profiles of serum complement C4 proteins in pediatric systemic lupus erythematosus (SLE) patients: tight associations of complement C4 and C3 protein levels in SLE but not in healthy subjects","volume":"586","author":"Wu","year":"2006","journal-title":"Adv. Exp. Med. Biol."},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib73","doi-asserted-by":"crossref","first-page":"1272","DOI":"10.1177\/0961203310371154","article-title":"The complex nature of serum C3 and C4 as biomarkers of lupus renal flare","volume":"19","author":"Birmingham","year":"2010","journal-title":"Lupus"},{"key":"10.1016\/j.jaut.2022.102979_bib74","doi-asserted-by":"crossref","first-page":"171","DOI":"10.2174\/1874312901812010171","article-title":"Causative, concomitant or coincidental?","volume":"12","author":"Sandhu","year":"2018","journal-title":"Open Rheumatol. J."},{"issue":"9814","key":"10.1016\/j.jaut.2022.102979_bib75","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1016\/S0140-6736(11)60935-5","article-title":"Hereditary angio-oedema","volume":"379","author":"Longhurst","year":"2012","journal-title":"Lancet"},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib76","doi-asserted-by":"crossref","first-page":"3596","DOI":"10.1002\/art.20561","article-title":"Measurement of erythrocyte C4d and complement receptor 1 in systemic lupus erythematosus","volume":"50","author":"Manzi","year":"2004","journal-title":"Arthritis Rheum."},{"issue":"9","key":"10.1016\/j.jaut.2022.102979_bib77","doi-asserted-by":"crossref","first-page":"1599","DOI":"10.1136\/annrheumdis-2015-207762","article-title":"Gene copy-number variations (CNVs) of complement C4 and C4A deficiency in genetic risk and pathogenesis of juvenile dermatomyositis","volume":"75","author":"Lintner","year":"2016","journal-title":"Ann. Rheum. Dis."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib78","first-page":"1","article-title":"Cell-bound complement activation products in systemic lupus erythematosus: comparison with anti-double-stranded DNA and standard complement measurements","author":"Putterman","year":"2014","journal-title":"Lupus Sci Med"},{"key":"10.1016\/j.jaut.2022.102979_bib79","article-title":"MI. Low copy numbers of complement C4 and C4A deficiency are risk factors for myositis, its subgroups and autoantibodies","author":"Zhou","year":"2022","journal-title":"Ann. Rheum. Dis."},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib80","doi-asserted-by":"crossref","first-page":"4429","DOI":"10.4049\/jimmunol.156.11.4429","article-title":"IFN-gamma up-regulates expression of the complement components C3 and C4 by stabilization of mRNA","volume":"156","author":"Mitchell","year":"1996","journal-title":"J. Immunol."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib81","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1016\/S1567-5769(01)00019-4","article-title":"Genetic, structural and functional diversities of human complement components C4A and C4B and their mouse homologues, Slp and C4","volume":"1","author":"Blanchong","year":"2001","journal-title":"Int. Immunopharm."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib82","doi-asserted-by":"crossref","first-page":"2734","DOI":"10.4049\/jimmunol.171.5.2734","article-title":"Diversity in intrinsic strengths of the human complement system: serum C4 protein concentrations correlate with C4 gene size and polygenic variations, hemolytic activities, and body mass index","volume":"171","author":"Yang","year":"2003","journal-title":"J. Immunol."},{"issue":"7","key":"10.1016\/j.jaut.2022.102979_bib83","doi-asserted-by":"crossref","first-page":"1289","DOI":"10.1016\/j.molimm.2008.11.018","article-title":"Great genotypic and phenotypic diversities associated with copy-number variations of complement C4 and RP-C4-CYP21-TNX (RCCX) modules: a comparison of Asian-Indian and European American populations","volume":"46","author":"Saxena","year":"2009","journal-title":"Mol. Immunol."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib84","doi-asserted-by":"crossref","first-page":"44","DOI":"10.2174\/1573399814666180417122030","article-title":"Complement components, C3 and C4, and the metabolic syndrome","volume":"15","author":"Copenhaver","year":"2019","journal-title":"Curr. Diabetes Rev."},{"issue":"22","key":"10.1016\/j.jaut.2022.102979_bib85","doi-asserted-by":"crossref","first-page":"2110","DOI":"10.1056\/NEJMra1100359","article-title":"Systemic lupus erythematosus","volume":"365","author":"Tsokos","year":"2011","journal-title":"N. Engl. J. Med."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib86","doi-asserted-by":"crossref","first-page":"986","DOI":"10.1073\/pnas.1616998114","article-title":"Structure and activation of C1, the complex initiating the classical pathway of the complement cascade","volume":"114","author":"Mortensen","year":"2017","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib87","doi-asserted-by":"crossref","first-page":"4369","DOI":"10.4049\/jimmunol.1101749","article-title":"Functional complement C1q abnormality leads to impaired immune complexes and apoptotic cell clearance","volume":"187","author":"Roumenina","year":"2011","journal-title":"J. Immunol."},{"key":"10.1016\/j.jaut.2022.102979_bib88","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1016\/S0065-2776(01)76021-X","article-title":"Systemic lupus erythematosus, complement deficiency, and apoptosis","volume":"76","author":"Pickering","year":"2000","journal-title":"Adv. Immunol."},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib89","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1038\/gene.2011.39","article-title":"Molecular basis of hereditary C1q deficiency\u2013revisited: identification of several novel disease-causing mutations","volume":"12","author":"Schejbel","year":"2011","journal-title":"Gene Immun."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib90","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1186\/1546-0096-11-41","article-title":"The identification of a novel splicing mutation in C1qB in a Japanese family with C1q deficiency: a case report","volume":"11","author":"Higuchi","year":"2013","journal-title":"Pediatr Rheumatol Online J"},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib91","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/j.imbio.2013.10.010","article-title":"New C1q mutation in a Tunisian family","volume":"219","author":"Jlajla","year":"2014","journal-title":"Immunobiology"},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib92","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/j.clim.2007.03.547","article-title":"C1q deficiency in an Inuit family: identification of a new class of C1q disease-causing mutations","volume":"124","author":"Marquart","year":"2007","journal-title":"Clin. Immunol."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib93","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1007\/BF00376120","article-title":"A homozygous point mutation results in a stop codon in the C1q B-chain of a C1q-deficient individual","volume":"27","author":"McAdam","year":"1988","journal-title":"Immunogenetics"},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib94","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1016\/j.imbio.2014.10.005","article-title":"Identification of a novel non-coding mutation in C1qB in a Dutch child with C1q deficiency associated with recurrent infections","volume":"220","author":"van Schaarenburg","year":"2015","journal-title":"Immunobiology"},{"issue":"10","key":"10.1016\/j.jaut.2022.102979_bib95","doi-asserted-by":"crossref","first-page":"1113","DOI":"10.1177\/0961203312443993","article-title":"Identification of novel coding mutation in C1qA gene in an African-American pedigree with lupus and C1q deficiency","volume":"21","author":"Namjou","year":"2012","journal-title":"Lupus"},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib96","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1093\/rheumatology\/kep387","article-title":"SLE with C1q deficiency treated with fresh frozen plasma: a 10-year experience","volume":"49","author":"Mehta","year":"2009","journal-title":"Rheumatology"},{"key":"10.1016\/j.jaut.2022.102979_bib97","doi-asserted-by":"crossref","first-page":"583853","DOI":"10.3389\/fimmu.2020.583853","article-title":"Novel postulates for therapeutic options","volume":"11","author":"Hosszu","year":"2020","journal-title":"Front. Immunol."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib98","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1084\/jem.194.6.781","article-title":"C1q and mannose binding lectin engagement of cell surface calreticulin and CD91 initiates macropinocytosis and uptake of apoptotic cells","volume":"194","author":"Ogden","year":"2001","journal-title":"J. Exp. Med."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib99","doi-asserted-by":"crossref","first-page":"2329","DOI":"10.4049\/jimmunol.180.4.2329","article-title":"C1q binds phosphatidylserine and likely acts as a multiligand-bridging molecule in apoptotic cell recognition","volume":"180","author":"Pa\u00efdassi","year":"2008","journal-title":"J. Immunol."},{"issue":"7","key":"10.1016\/j.jaut.2022.102979_bib100","doi-asserted-by":"crossref","first-page":"3978","DOI":"10.4049\/jimmunol.169.7.3978","article-title":"Role of surfactant proteins A, D, and C1q in the clearance of apoptotic cells in vivo and in vitro: calreticulin and CD91 as a common collectin receptor complex","volume":"169","author":"Vandivier","year":"2002","journal-title":"J. Immunol."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib101","doi-asserted-by":"crossref","first-page":"1543","DOI":"10.1002\/art.21783","article-title":"Impaired recognition of apoptotic neutrophils by the C1q\/calreticulin and CD91 pathway in systemic lupus erythematosus","volume":"54","author":"Donnelly","year":"2006","journal-title":"Arthritis Rheum."},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib102","doi-asserted-by":"crossref","first-page":"4738","DOI":"10.4049\/jimmunol.1001731","article-title":"C1q deficiency leads to the defective suppression of IFN-alpha in response to nucleoprotein containing immune complexes","volume":"185","author":"Santer","year":"2010","journal-title":"J. Immunol."},{"issue":"S1","key":"10.1016\/j.jaut.2022.102979_bib103","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1096\/fasebj.22.1_supplement.673.1","article-title":"C1q is a molecular switch dictating the monocyte to dendritic cell (DC) transition and arrests DCs in an immature phenotype","volume":"22","author":"Hosszu","year":"2008","journal-title":"Faseb. J."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib104","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1177\/1753425909339815","article-title":"Evidence that a C1q\/C1qR system regulates monocyte-derived dendritic cell differentiation at the interface of innate and acquired immunity","volume":"16","author":"Hosszu","year":"2010","journal-title":"Innate Immun."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib105","doi-asserted-by":"crossref","first-page":"3512","DOI":"10.4049\/jimmunol.0803573","article-title":"Autoantibodies against complement C1q specifically target C1q bound on early apoptotic cells","volume":"183","author":"Bigler","year":"2009","journal-title":"J. Immunol."},{"key":"10.1016\/j.jaut.2022.102979_bib106","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1196\/annals.1422.025","article-title":"Autoantibodies against protective molecules\u2013C1q, C-reactive protein, serum amyloid P, mannose-binding lectin, and apolipoprotein A1: prevalence in systemic lupus erythematosus","volume":"1108","author":"Shoenfeld","year":"2007","journal-title":"Ann. N. Y. Acad. Sci."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib107","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1177\/0961203317741565","article-title":"Systemic lupus erythematosus with C1q deficiency: treatment with fresh frozen plasma","volume":"27","author":"Ekinci","year":"2018","journal-title":"Lupus"},{"issue":"12","key":"10.1016\/j.jaut.2022.102979_bib108","doi-asserted-by":"crossref","first-page":"3713","DOI":"10.1002\/eji.200425616","article-title":"Restoration of C1q levels by bone marrow transplantation attenuates autoimmune disease associated with C1q deficiency in mice","volume":"34","author":"Cortes-Hernandez","year":"2004","journal-title":"Eur. J. Immunol."},{"issue":"7","key":"10.1016\/j.jaut.2022.102979_bib109","doi-asserted-by":"crossref","first-page":"4033","DOI":"10.4049\/jimmunol.167.7.4033","article-title":"Reconstitution of the complement function in C1q-deficient (C1qa-\/-) mice with wild-type bone marrow cells","volume":"167","author":"Petry","year":"2001","journal-title":"J. Immunol."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib110","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/j.jaci.2013.07.035","article-title":"Successful cure of C1q deficiency in human subjects treated with hematopoietic stem cell transplantation","volume":"133","author":"Arkwright","year":"2014","journal-title":"J. Allergy Clin. Immunol."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib111","doi-asserted-by":"crossref","first-page":"1356","DOI":"10.1097\/TP.0000000000000975","article-title":"Allogeneic hematopoietic stem cell transplantation in the treatment of human C1q deficiency: the karolinska experience","volume":"100","author":"Olsson","year":"2016","journal-title":"Transplantation"},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib112","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1172\/JCI106902","article-title":"C1r deficiency: an inborn error associated with cutaneous and renal disease","volume":"51","author":"Day","year":"1972","journal-title":"J. Clin. Invest."},{"issue":"13","key":"10.1016\/j.jaut.2022.102979_bib113","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1056\/NEJM197203302861304","article-title":"Lupus-erythematosus-like syndrome with a familial defect of complement","volume":"286","author":"Moncada","year":"1972","journal-title":"N. Engl. J. Med."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib114","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1084\/jem.131.4.803","article-title":"Deficiency of C1r in human serum. Effects on the structure and function of macromolecular C1","volume":"131","author":"Pickering","year":"1970","journal-title":"J. Exp. Med."},{"issue":"12","key":"10.1016\/j.jaut.2022.102979_bib115","doi-asserted-by":"crossref","first-page":"7612","DOI":"10.4049\/jimmunol.166.12.7612","article-title":"Molecular basis of a selective C1s deficiency associated with early onset multiple autoimmune diseases","volume":"166","author":"Dragon-Durey","year":"2001","journal-title":"J. Immunol."},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib116","doi-asserted-by":"crossref","first-page":"1126","DOI":"10.1177\/0961203311404914","article-title":"Clinical presentations and molecular basis of complement C1r deficiency in a male African-American patient with systemic lupus erythematosus","volume":"20","author":"Wu","year":"2011","journal-title":"Lupus"},{"issue":"9","key":"10.1016\/j.jaut.2022.102979_bib117","doi-asserted-by":"crossref","first-page":"1832","DOI":"10.1002\/art.40158","article-title":"Brief report: deficiency of complement 1r subcomponent in early-onset systemic lupus erythematosus: the role of disease-modifying alleles in a monogenic disease","volume":"69","author":"Demirkaya","year":"2017","journal-title":"Arthritis Rheumatol."},{"issue":"12","key":"10.1016\/j.jaut.2022.102979_bib118","doi-asserted-by":"crossref","first-page":"2917","DOI":"10.1002\/art.39781","article-title":"A novel mechanism for generating the interferon signature in lupus: opsonization of dead cells by complement and IgM","volume":"68","author":"Zhuang","year":"2016","journal-title":"Arthritis Rheumatol."},{"issue":"13","key":"10.1016\/j.jaut.2022.102979_bib119","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1056\/NEJM199203263261306","article-title":"Molecular heterogeneity of C2 deficiency","volume":"326","author":"Johnson","year":"1992","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.jaut.2022.102979_bib120","series-title":"Systemic Lupus Erythematosus, a Companion to Rheumatology","first-page":"183","article-title":"Complement deficiencies in human systemic lupus erythematosus (SLE) and SLE nephritis: epidemiology and pathogenesis","author":"Yu","year":"2007"},{"issue":"10","key":"10.1016\/j.jaut.2022.102979_bib121","doi-asserted-by":"crossref","first-page":"5824","DOI":"10.1074\/jbc.271.10.5824","article-title":"Type II human complement C2 deficiency. Allele-specific amino acid substitutions (Ser189--> Phe; Gly444--> Arg) cause impaired C2 secretion","volume":"271","author":"Wetsel","year":"1996","journal-title":"J. Biol. Chem."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib122","doi-asserted-by":"crossref","first-page":"578","DOI":"10.4049\/jimmunol.161.2.578","article-title":"A novel type II complement C2 deficiency allele in an African-American family","volume":"161","author":"Zhu","year":"1998","journal-title":"J. Immunol."},{"issue":"13","key":"10.1016\/j.jaut.2022.102979_bib123","doi-asserted-by":"crossref","first-page":"9347","DOI":"10.1016\/S0021-9258(19)50430-6","article-title":"Type I human complement C2 deficiency. A 28-base pair gene deletion causes skipping of exon 6 during RNA splicing","volume":"267","author":"Johnson","year":"1992","journal-title":"J. Biol. Chem."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib124","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1046\/j.1365-2567.1998.00392.x","article-title":"Molecular heterogeneity in deficiency of complement protein C2 type I","volume":"93","author":"Wang","year":"1998","journal-title":"Immunology"},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib125","doi-asserted-by":"crossref","first-page":"1158","DOI":"10.1172\/JCI114821","article-title":"Molecular basis of hereditary C3 deficiency","volume":"86","author":"Botto","year":"1990","journal-title":"J. Clin. Invest."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib126","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1111\/j.1365-3083.2006.01729.x","article-title":"Clinical aspects and molecular basis of primary deficiencies of complement component C3 and its regulatory proteins factor I and factor H","volume":"63","author":"E SR","year":"2006","journal-title":"Scand. J. Immunol."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib127","doi-asserted-by":"crossref","first-page":"298","DOI":"10.1016\/j.bbrc.2005.02.159","article-title":"Molecular analysis of a novel hereditary C3 deficiency with systemic lupus erythematosus","volume":"330","author":"Tsukamoto","year":"2005","journal-title":"Biochem. Biophys. Res. Commun."},{"issue":"5948","key":"10.1016\/j.jaut.2022.102979_bib128","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1038\/307237a0","article-title":"A molecular map of the human major histocompatibility complex class III region linking complement genes C4, C2 and factor B","volume":"307","author":"Carroll","year":"1984","journal-title":"Nature"},{"key":"10.1016\/j.jaut.2022.102979_bib129","doi-asserted-by":"crossref","first-page":"D927","DOI":"10.2741\/Yang","article-title":"Features of the two gene pairs RD-SKI2W and DOM3Z-RP1 located between complement component genes factor B and C4 at the MHC class III region","volume":"6","author":"Yang","year":"2001","journal-title":"Front. Biosci."},{"issue":"7","key":"10.1016\/j.jaut.2022.102979_bib130","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1016\/S0167-5699(00)01664-9","article-title":"The human and mouse MHC class III region: a parade of 21 genes at the centromeric segment","volume":"21","author":"Yu","year":"2000","journal-title":"Immunol. Today"},{"issue":"12","key":"10.1016\/j.jaut.2022.102979_bib131","doi-asserted-by":"crossref","first-page":"2183","DOI":"10.1084\/jem.191.12.2183","article-title":"Deficiencies of human complement component C4A and C4B and heterozygosity in length variants of RP-C4-CYP21-TNX (RCCX) modules in caucasians. The load of RCCX genetic diversity on major histocompatibility complex-associated disease","volume":"191","author":"Blanchong","year":"2000","journal-title":"J. Exp. Med."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib132","doi-asserted-by":"crossref","first-page":"1442","DOI":"10.1002\/art.39589","article-title":"Effects of complement C4 gene copy number variations, size dichotomy, and C4A deficiency on genetic risk and clinical presentation of systemic lupus erythematosus in East Asian populations","volume":"68","author":"Chen","year":"2016","journal-title":"Arthritis Rheumatol."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib133","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1086\/342777","article-title":"Genetic sophistication of human complement components C4A and C4B and RP-C4-CYP21-TNX (RCCX) modules in the major histocompatibility complex","volume":"71","author":"Chung","year":"2002","journal-title":"Am. J. Hum. Genet."},{"issue":"17","key":"10.1016\/j.jaut.2022.102979_bib134","doi-asserted-by":"crossref","first-page":"12147","DOI":"10.1074\/jbc.274.17.12147","volume":"274","author":"Yang","year":"1999","journal-title":"J. Biol. Chem."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib135","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1007\/BF00396104","article-title":"Definitive RFLPs to distinguish between the human complement C4A\/C4B isotypes and the major Rodgers\/Chido determinants: application to the study of C4 null alleles","volume":"25","author":"Yu","year":"1987","journal-title":"Immunogenetics"},{"issue":"9","key":"10.1016\/j.jaut.2022.102979_bib136","first-page":"8","article-title":"An RNA metabolism and surveillance quartet in the major histocompatibility complex","author":"Zhou","year":"2019","journal-title":"Cells"},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib137","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1007\/BF00177825","article-title":"The dichotomous size variation of human complement C4 genes is mediated by a novel family of endogenous retroviruses, which also establishes species-specific genomic patterns among Old World primates","volume":"40","author":"Dangel","year":"1994","journal-title":"Immunogenetics"},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib138","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.4049\/jimmunol.146.3.1057","article-title":"The complete exon-intron structure of a human complement component C4A gene. DNA sequences, polymorphism, and linkage to the 21-hydroxylase gene","volume":"146","author":"Yu","year":"1991","journal-title":"J. Immunol."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib139","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1007\/s00251-004-0705-y","article-title":"Detection of retroviral antisense transcripts and promoter activity of the HERV-K(C4) insertion in the MHC class III region","volume":"56","author":"Mack","year":"2004","journal-title":"Immunogenetics"},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib140","doi-asserted-by":"crossref","first-page":"2873","DOI":"10.1002\/j.1460-2075.1986.tb04582.x","article-title":"Structural basis of the polymorphism of human complement components C4A and C4B: gene size, reactivity and antigenicity","volume":"5","author":"Yu","year":"1986","journal-title":"EMBO J."},{"issue":"12","key":"10.1016\/j.jaut.2022.102979_bib141","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1016\/j.it.2004.10.006","article-title":"Sex, MHC and complement C4 in autoimmune diseases","volume":"25","author":"Yu","year":"2004","journal-title":"Trends Immunol."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib142","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s002510000288","article-title":"The endogenous retroviral insertion in the human complement C4 gene modulates the expression of homologous genes by antisense inhibition","volume":"53","author":"Schneider","year":"2001","journal-title":"Immunogenetics"},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib143","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1007\/BF00364425","article-title":"A structural model for the location of the Rodgers and the Chido antigenic determinants and their correlation with the human complement component C4A\/C4B isotypes","volume":"27","author":"Yu","year":"1988","journal-title":"Immunogenetics"},{"key":"10.1016\/j.jaut.2022.102979_bib144","doi-asserted-by":"crossref","DOI":"10.1002\/0471142735.im1308s68","article-title":"Human complement components C4A and C4B genetic diversities: complex genotypes and phenotypes","author":"Chung","year":"2005","journal-title":"Curr. Protoc. Im."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib145","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1086\/342778","article-title":"Determining the one, two, three, or four long and short loci of human complement C4 in a major histocompatibility complex haplotype encoding C4A or C4B proteins","volume":"71","author":"Chung","year":"2002","journal-title":"Am. J. Hum. Genet."},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib146","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1016\/S0198-8859(98)00068-8","article-title":"Characterization of non-expressed C4 genes in a case of complete C4 deficiency: identification of a novel point mutation leading to a premature stop codon","volume":"59","author":"Fredrikson","year":"1998","journal-title":"Hum. Immunol."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib147","doi-asserted-by":"crossref","first-page":"3687","DOI":"10.4049\/jimmunol.162.6.3687","article-title":"Deficiency of human complement protein C4 due to identical frameshift mutations in the C4A and C4B genes","volume":"162","author":"Lokki","year":"1999","journal-title":"J. Immunol."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib148","doi-asserted-by":"crossref","first-page":"1570","DOI":"10.4049\/jimmunol.169.3.1570","article-title":"The molecular basis of complete complement C4A and C4B deficiencies in a systemic lupus erythematosus patient with homozygous C4A and C4B mutant genes","volume":"169","author":"Rupert","year":"2002","journal-title":"J. Immunol."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib149","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1038\/gene.2009.10","article-title":"Molecular basis of complete complement C4 deficiency in two North-African families with systemic lupus erythematosus","volume":"10","author":"Wu","year":"2009","journal-title":"Gene Immun."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib150","doi-asserted-by":"crossref","first-page":"2803","DOI":"10.4049\/jimmunol.173.4.2803","article-title":"Complete complement components C4A and C4B deficiencies in human kidney diseases and systemic lupus erythematosus","volume":"173","author":"Yang","year":"2004","journal-title":"J. Immunol."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib151","doi-asserted-by":"crossref","first-page":"1681","DOI":"10.1172\/JCI116377","article-title":"Genetic basis of human complement C4A deficiency. Detection of a point mutation leading to nonexpression","volume":"91","author":"Barba","year":"1993","journal-title":"J. Clin. Invest."},{"issue":"10","key":"10.1016\/j.jaut.2022.102979_bib152","first-page":"2144","article-title":"C4A deficiency due to a 2 bp insertion is increased in patients with systemic lupus erythematosus","volume":"26","author":"Sullivan","year":"1999","journal-title":"J. Rheumatol."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib153","doi-asserted-by":"crossref","first-page":"1037","DOI":"10.1086\/518257","volume":"80","author":"Yang","year":"2007","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.jaut.2022.102979_bib154","doi-asserted-by":"crossref","first-page":"739430","DOI":"10.3389\/fimmu.2021.739430","article-title":"Human complement C4B allotypes and deficiencies in selected cases with autoimmune diseases","volume":"12","author":"Zhou","year":"2021","journal-title":"Front. Immunol."},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib155","doi-asserted-by":"crossref","first-page":"1819","DOI":"10.1002\/j.1460-2075.1984.tb02052.x","article-title":"A comparison of the properties of two classes, C4A and C4B, of the human complement component C4","volume":"3","author":"Law","year":"1984","journal-title":"EMBO J."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib156","doi-asserted-by":"crossref","first-page":"3019","DOI":"10.4049\/jimmunol.132.6.3019","article-title":"The molecular basis for the difference in immune hemolysis activity of the Chido and Rodgers isotypes of human complement component C4","volume":"132","author":"Isenman","year":"1984","journal-title":"J. Immunol."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib157","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1084\/jem.192.3.359","article-title":"A hierarchical role for classical pathway complement proteins in the clearance of apoptotic cells in vivo","volume":"192","author":"Taylor","year":"2000","journal-title":"J. Exp. Med."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib158","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1016\/S1471-4906(03)00030-9","article-title":"Recognition and clearance of apoptotic cells: a role for complement and pentraxins","volume":"24","author":"Nauta","year":"2003","journal-title":"Trends Immunol."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib159","first-page":"13","article-title":"Clinical features of patients with homozygous complement C4A or C4B deficiency","author":"Liesmaa","year":"2018","journal-title":"PLoS One"},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib160","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1016\/j.immuni.2006.08.010","article-title":"Type I interferon in systemic lupus erythematosus and other autoimmune diseases","volume":"25","author":"Banchereau","year":"2006","journal-title":"Immunity"},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib161","doi-asserted-by":"crossref","first-page":"1815","DOI":"10.1002\/art.24555","article-title":"Elevated serum interferon-alpha activity in juvenile dermatomyositis: associations with disease activity at diagnosis and after thirty-six months of therapy","volume":"60","author":"Niewold","year":"2009","journal-title":"Arthritis Rheum."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib162","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1177\/0961203317735187","article-title":"Low copy numbers of complement C4 and homozygous deficiency of C4A may predispose to severe disease and earlier disease onset in patients with systemic lupus erythematosus","volume":"27","author":"Juptner","year":"2018","journal-title":"Lupus"},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib163","doi-asserted-by":"crossref","first-page":"3012","DOI":"10.4049\/jimmunol.179.5.3012","volume":"179","author":"Wu","year":"2007","journal-title":"J. Immunol."},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib164","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1177\/0961203306072311","article-title":"Ethnic and geographical differences in systemic lupus erythematosus: an overview","volume":"15","author":"Lau","year":"2006","journal-title":"Lupus"},{"issue":"4\u20135","key":"10.1016\/j.jaut.2022.102979_bib165","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1111\/j.1744-313X.1987.tb00383.x","article-title":"Deficiency of C4A is a genetic determinant of systemic lupus erythematosus in three ethnic groups","volume":"14","author":"Dunckley","year":"1987","journal-title":"J. Immunogenet"},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib166","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.1002\/art.37854","article-title":"Deletion variants of RABGAP1L, 10q21.3, and C4 are associated with the risk of systemic lupus erythematosus in Korean women","volume":"65","author":"Kim","year":"2013","journal-title":"Arthritis Rheum."},{"issue":"10","key":"10.1016\/j.jaut.2022.102979_bib167","doi-asserted-by":"crossref","first-page":"3047","DOI":"10.1007\/s00296-011-2023-7","article-title":"Confirmation of C4 gene copy number variation and the association with systemic lupus erythematosus in Chinese Han population","volume":"32","author":"Lv","year":"2012","journal-title":"Rheumatol. Int."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib168","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1186\/s42358-019-0076-6","article-title":"Impact of C4, C4A and C4B gene copy number variation in the susceptibility, phenotype and progression of systemic lupus erythematosus","volume":"59","author":"Pereira","year":"2019","journal-title":"Adv Rheumatol"},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib169","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1093\/rheumatology\/kev436","article-title":"Low C4, C4A and C4B gene copy numbers are stronger risk factors for juvenile-onset than for adult-onset systemic lupus erythematosus","volume":"55","author":"Pereira","year":"2016","journal-title":"Rheumatology"},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib170","doi-asserted-by":"crossref","DOI":"10.1136\/lupus-2019-000333","article-title":"Elevated serum complement levels and higher gene copy number of complement C4B are associated with hypertension and effective response to statin therapy in childhood-onset systemic lupus erythematosus (SLE)","volume":"6","author":"Mulvihill","year":"2019","journal-title":"Lupus Sci Med"},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib171","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1002\/art.30645","article-title":"Atherosclerosis Prevention in Pediatric Lupus Erythematosus I. Use of atorvastatin in systemic lupus erythematosus in children and adolescents","volume":"64","author":"Schanberg","year":"2012","journal-title":"Arthritis Rheum."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib172","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1001\/archderm.1953.01540080003001","article-title":"Complement in disseminated (systemic) lupus erythematosus","volume":"68","author":"Elliott","year":"1953","journal-title":"AMA Arch Derm Syphilol"},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib173","doi-asserted-by":"crossref","first-page":"555","DOI":"10.7326\/0003-4819-75-4-555","article-title":"Serum complement component levels in human glomerulonephritis","volume":"75","author":"Lewis","year":"1971","journal-title":"Ann. Intern. Med."},{"issue":"10","key":"10.1016\/j.jaut.2022.102979_bib174","doi-asserted-by":"crossref","first-page":"3087","DOI":"10.1002\/art.21305","article-title":"Reticulocytes bearing C4d as biomarkers of disease activity for systemic lupus erythematosus","volume":"52","author":"Liu","year":"2005","journal-title":"Arthritis Rheum."},{"key":"10.1016\/j.jaut.2022.102979_bib175","doi-asserted-by":"crossref","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."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib176","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1111\/j.1538-7836.2006.01753.x","article-title":"International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS)","volume":"4","author":"Miyakis","year":"2006","journal-title":"J. Thromb. Haemostasis"},{"issue":"7","key":"10.1016\/j.jaut.2022.102979_bib177","doi-asserted-by":"crossref","first-page":"1309","DOI":"10.1002\/1529-0131(199907)42:7<1309::AID-ANR1>3.0.CO;2-F","article-title":"International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop","volume":"42","author":"Wilson","year":"1999","journal-title":"Arthritis Rheum."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib178","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1111\/1756-185X.12438","article-title":"Antiphospholipid syndrome: 30 years and our contribution","volume":"18","author":"Koike","year":"2015","journal-title":"Int J Rheum Dis"},{"issue":"Pt 2","key":"10.1016\/j.jaut.2022.102979_bib179","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1042\/bj2770387","article-title":"Complete nucleotide and deduced amino acid sequence of human beta 2-glycoprotein I","volume":"277","author":"Steinkasserer","year":"1991","journal-title":"Biochem. J."},{"key":"10.1016\/j.jaut.2022.102979_bib180","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1016\/j.freeradbiomed.2018.03.048","article-title":"Oxidative post-translational modification of betaeta 2-glycoprotein I in the pathophysiology of the anti-phospholipid syndrome","volume":"125","author":"Weaver","year":"2018","journal-title":"Free Radic. Biol. Med."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib181","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1136\/annrheumdis-2017-212224","article-title":"Complement activation predicts adverse pregnancy outcome in patients with systemic lupus erythematosus and\/or antiphospholipid antibodies","volume":"77","author":"Kim","year":"2018","journal-title":"Ann. Rheum. Dis."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib182","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1016\/j.ajog.2006.10.879","article-title":"Excessive complement activation is associated with placental injury in patients with antiphospholipid antibodies","volume":"196","author":"Shamonki","year":"2007","journal-title":"Am. J. Obstet. Gynecol."},{"key":"10.1016\/j.jaut.2022.102979_bib183","doi-asserted-by":"crossref","first-page":"S43","DOI":"10.1016\/S0049-3848(17)30066-X","article-title":"Antiphospholipid syndrome","volume":"1","author":"Cervera","year":"2017","journal-title":"Thromb. Res."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib184","doi-asserted-by":"crossref","first-page":"1019","DOI":"10.1002\/art.10187","article-title":"Euro-Phospholipid Project G. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients","volume":"46","author":"Cervera","year":"2002","journal-title":"Arthritis Rheum."},{"issue":"9","key":"10.1016\/j.jaut.2022.102979_bib185","doi-asserted-by":"crossref","first-page":"682","DOI":"10.7326\/0003-4819-112-9-682","article-title":"Antiphospholipid antibodies: anticardiolipin and the lupus anticoagulant in systemic lupus erythematosus (SLE) and in non-SLE disorders. Prevalence and clinical significance","volume":"112","author":"Love","year":"1990","journal-title":"Ann. Intern. Med."},{"key":"10.1016\/j.jaut.2022.102979_bib186","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.jaut.2016.10.004","article-title":"The antiphospholipid syndrome in patients with systemic lupus erythematosus","volume":"76","author":"Pons-Estel","year":"2017","journal-title":"J. Autoimmun."},{"key":"10.1016\/j.jaut.2022.102979_bib187","doi-asserted-by":"crossref","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","volume":"10","author":"Savelli","year":"2019","journal-title":"Front. Immunol."},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib188","doi-asserted-by":"crossref","first-page":"1639","DOI":"10.1172\/JCI20309","article-title":"Complement system on the attack in autoimmunity","volume":"112","author":"Atkinson","year":"2003","journal-title":"J. Clin. Invest."},{"issue":"1 Pt 1","key":"10.1016\/j.jaut.2022.102979_bib189","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1016\/S0002-9378(11)90700-5","article-title":"Immunoglobulin G fractions from patients with antiphospholipid antibodies cause fetal death in BALB\/c mice: a model for autoimmune fetal loss","volume":"163","author":"Branch","year":"1990","journal-title":"Am. J. Obstet. Gynecol."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib190","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1002\/path.2893","article-title":"Classical complement activation as a footprint for murine and human antiphospholipid antibody-induced fetal loss","volume":"225","author":"Cohen","year":"2011","journal-title":"J. Pathol."},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib191","doi-asserted-by":"crossref","first-page":"1644","DOI":"10.1172\/JCI200318817","article-title":"Complement C5a receptors and neutrophils mediate fetal injury in the antiphospholipid syndrome","volume":"112","author":"Girardi","year":"2003","journal-title":"J. Clin. Invest."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib192","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1084\/jem.200116116","article-title":"Complement C3 activation is required for antiphospholipid antibody-induced fetal loss","volume":"195","author":"Holers","year":"2002","journal-title":"J. Exp. Med."},{"key":"10.1016\/j.jaut.2022.102979_bib193","doi-asserted-by":"crossref","first-page":"1388","DOI":"10.3389\/fimmu.2018.01388","article-title":"Pathogenic role of complement in antiphospholipid syndrome and therapeutic implications","volume":"9","author":"Tedesco","year":"2018","journal-title":"Front. Immunol."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib194","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1111\/imr.12471","article-title":"Dangerous liaisons: complement, coagulation, and kallikrein\/kinin cross-talk act as a linchpin in the events leading to thromboinflammation","volume":"274","author":"Ekdahl","year":"2016","journal-title":"Immunol. Rev."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib195","doi-asserted-by":"crossref","first-page":"1030","DOI":"10.1136\/ard.2008.090670","article-title":"Complement activation in patients with primary antiphospholipid syndrome","volume":"68","author":"Oku","year":"2009","journal-title":"Ann. Rheum. Dis."},{"issue":"10","key":"10.1016\/j.jaut.2022.102979_bib196","doi-asserted-by":"crossref","first-page":"1001","DOI":"10.1016\/j.autrev.2016.07.020","article-title":"Complement and thrombosis in the antiphospholipid syndrome","volume":"15","author":"Oku","year":"2016","journal-title":"Autoimmun. Rev."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib197","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/j.molimm.2013.05.009","article-title":"Autoantibodies against complement components and functional consequences","volume":"56","author":"Dragon-Durey","year":"2013","journal-title":"Mol. Immunol."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib198","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1177\/096120339600500309","article-title":"Autoantibodies to the collagen-like region of C1q are strongly associated with classical pathway-mediated hypocomplementemia in systemic lupus erythematosus","volume":"5","author":"Fremeaux-Bacchi","year":"1996","journal-title":"Lupus"},{"key":"10.1016\/j.jaut.2022.102979_bib199","doi-asserted-by":"crossref","first-page":"117","DOI":"10.3389\/fimmu.2013.00117","article-title":"Anti-C1q autoantibodies, novel tests, and clinical consequences","volume":"4","author":"Mahler","year":"2013","journal-title":"Front. Immunol."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib200","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1177\/0961203314547791","article-title":"Anti-C1q antibodies in systemic lupus erythematosus","volume":"24","author":"Orbai","year":"2015","journal-title":"Lupus"},{"key":"10.1016\/j.jaut.2022.102979_bib201","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1016\/S0065-2776(08)60527-1","article-title":"Conglutinin and immunoconglutinins","volume":"6","author":"Lachmann","year":"1967","journal-title":"Adv. Immunol."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib202","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1111\/j.1365-2249.1992.tb07937.x","article-title":"Detection and characterization of immunoconglutinins in patients with systemic lupus erythematosus (SLE): serial analysis in relation to disease course","volume":"90","author":"Nilsson","year":"1992","journal-title":"Clin. Exp. Immunol."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib203","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1006\/jaut.1997.0147","article-title":"Correlation between anti-C1q and immune conglutinin levels, but not between levels of antibodies to the structurally related autoantigens C1q and type II collagen in SLE or RA","volume":"10","author":"Ronnelid","year":"1997","journal-title":"J. Autoimmun."},{"issue":"42","key":"10.1016\/j.jaut.2022.102979_bib204","doi-asserted-by":"crossref","first-page":"25343","DOI":"10.1074\/jbc.M115.647008","article-title":"Functional characterization of autoantibodies against complement component C3 in patients with lupus nephritis","volume":"290","author":"Vasilev","year":"2015","journal-title":"J. Biol. Chem."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib205","doi-asserted-by":"crossref","first-page":"47","DOI":"10.2215\/CJN.03990415","article-title":"Relationship of circulating anti-C3b and anti-C1q IgG to lupus nephritis and its flare","volume":"11","author":"Birmingham","year":"2016","journal-title":"Clin. J. Am. Soc. Nephrol."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib206","first-page":"449","article-title":"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","volume":"68","author":"Ito","year":"1989","journal-title":"Immunology"},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib207","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.imlet.2014.01.014","article-title":"Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome","volume":"160","author":"Jozsi","year":"2014","journal-title":"Immunol. Lett."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib208","doi-asserted-by":"crossref","first-page":"2051","DOI":"10.4049\/jimmunol.125.5.2051","article-title":"Relative resistance of the F-42-stabilized classical pathway C3 convertase to inactivation by C4-binding protein","volume":"125","author":"Daha","year":"1980","journal-title":"J. Immunol."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib209","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/j.clim.2012.09.007","article-title":"Autoantibody stabilization of the classical pathway C3 convertase leading to C3 deficiency and Neisserial sepsis: C4 nephritic factor revisited","volume":"145","author":"Miller","year":"2012","journal-title":"Clin. Immunol."},{"key":"10.1016\/j.jaut.2022.102979_bib210","article-title":"Low copy numbers of complement C4 and C4A deficiency are risk factors for myositis, its subgroups and autoantibodies","author":"Zhou","year":"2022","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.jaut.2022.102979_bib211","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.3389\/fimmu.2018.01057","article-title":"Complement in the initiation and evolution of rheumatoid arthritis","volume":"9","author":"Holers","year":"2018","journal-title":"Front. Immunol."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib212","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1002\/art.1780180603","article-title":"Identification of immunoglobulins and complement in rheumatoid articular collagenous tissues","volume":"18","author":"Cooke","year":"1975","journal-title":"Arthritis Rheum."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib213","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1136\/ard.58.3.175","article-title":"Complement C1s activation in degenerating articular cartilage of rheumatoid arthritis patients: immunohistochemical studies with an active form specific antibody","volume":"58","author":"Nakagawa","year":"1999","journal-title":"Ann. Rheum. Dis."},{"issue":"10","key":"10.1016\/j.jaut.2022.102979_bib214","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1016\/j.autrev.2011.04.012","article-title":"Role of the complement system in rheumatoid arthritis and psoriatic arthritis: relationship with anti-TNF inhibitors","volume":"10","author":"Ballanti","year":"2011","journal-title":"Autoimmun. Rev."},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib215","doi-asserted-by":"crossref","first-page":"2482","DOI":"10.4049\/jimmunol.2101170","article-title":"Analysis of complement gene expression, clinical associations, and biodistribution of complement proteins in the synovium of early rheumatoid arthritis patients reveals unique pathophysiologic features","volume":"208","author":"Banda","year":"2022","journal-title":"J. Immunol."},{"issue":"14","key":"10.1016\/j.jaut.2022.102979_bib216","doi-asserted-by":"crossref","first-page":"1707","DOI":"10.2174\/092986706777441959","article-title":"A review of current knowledge of the complement system and the therapeutic opportunities in inflammatory arthritis","volume":"13","author":"Mizuno","year":"2006","journal-title":"Curr. Med. Chem."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib217","first-page":"23","article-title":"Complement system in psoriatic arthritis: a useful marker in response prediction and monitoring of anti-TNF treatment","volume":"30","author":"Chimenti","year":"2012","journal-title":"Clin. Exp. Rheumatol."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib218","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1093\/rheumatology\/39.2.189","article-title":"Evidence for activation of the alternate complement pathway in patients with juvenile rheumatoid arthritis","volume":"39","author":"Aggarwal","year":"2000","journal-title":"Rheumatology"},{"key":"10.1016\/j.jaut.2022.102979_bib219","doi-asserted-by":"crossref","first-page":"177","DOI":"10.3389\/fimmu.2016.00177","article-title":"Immune complexes in juvenile idiopathic arthritis","volume":"7","author":"Moore","year":"2016","journal-title":"Front. Immunol."},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib220","doi-asserted-by":"crossref","first-page":"1359","DOI":"10.1002\/art.1780291108","article-title":"Complement activation in synovial fluid and tissue from patients with juvenile rheumatoid arthritis","volume":"29","author":"Mollnes","year":"1986","journal-title":"Arthritis Rheum."},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib221","doi-asserted-by":"crossref","first-page":"1338","DOI":"10.1002\/art.33472","article-title":"Increased frequency of complement C4B deficiency in rheumatoid arthritis","volume":"64","author":"Rigby","year":"2012","journal-title":"Arthritis Rheum."},{"issue":"10","key":"10.1016\/j.jaut.2022.102979_bib222","doi-asserted-by":"crossref","first-page":"1156","DOI":"10.1038\/ng.218","article-title":"Rheumatoid arthritis susceptibility loci at chromosomes 10p15, 12q13 and 22q13","volume":"40","author":"Barton","year":"2008","journal-title":"Nat. Genet."},{"issue":"9","key":"10.1016\/j.jaut.2022.102979_bib223","doi-asserted-by":"crossref","first-page":"e278","DOI":"10.1371\/journal.pmed.0040278","article-title":"A candidate gene approach identifies the TRAF1\/C5 region as a risk factor for rheumatoid arthritis","volume":"4","author":"Kurreeman","year":"2007","journal-title":"PLoS Med."},{"issue":"12","key":"10.1016\/j.jaut.2022.102979_bib224","doi-asserted-by":"crossref","first-page":"1431","DOI":"10.1038\/ng.2007.32","article-title":"Rheumatoid arthritis association at 6q23","volume":"39","author":"Thomson","year":"2007","journal-title":"Nat. Genet."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib225","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1111\/cei.12097","article-title":"Genetic variants in the region of the C1q genes are associated with rheumatoid arthritis","volume":"173","author":"Trouw","year":"2013","journal-title":"Clin. Exp. Immunol."},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib226","doi-asserted-by":"crossref","first-page":"1168","DOI":"10.1093\/rheumatology\/ken226","article-title":"Mannose-binding lectin polymorphisms are not associated with rheumatoid arthritis\u2014confirmation in two large cohorts","volume":"47","author":"van de Geijn","year":"2008","journal-title":"Rheumatology"},{"issue":"7","key":"10.1016\/j.jaut.2022.102979_bib227","doi-asserted-by":"crossref","first-page":"3029","DOI":"10.4049\/jimmunol.1402956","article-title":"Functional analysis of a complement polymorphism (rs17611) associated with rheumatoid arthritis","volume":"194","author":"Giles","year":"2015","journal-title":"J. Immunol."},{"issue":"12","key":"10.1016\/j.jaut.2022.102979_bib228","doi-asserted-by":"crossref","first-page":"1773","DOI":"10.1093\/rheumatology\/kem222","article-title":"Blocking the receptor for C5a in patients with rheumatoid arthritis does not reduce synovial inflammation","volume":"46","author":"Vergunst","year":"2007","journal-title":"Rheumatology"},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib229","doi-asserted-by":"crossref","first-page":"4340","DOI":"10.4049\/jimmunol.164.8.4340","article-title":"A role for complement in antibody-mediated inflammation: C5-deficient DBA\/1 mice are resistant to collagen-induced arthritis","volume":"164","author":"Wang","year":"2000","journal-title":"J. Immunol."},{"issue":"4","key":"10.1016\/j.jaut.2022.102979_bib230","doi-asserted-by":"crossref","first-page":"e95519","DOI":"10.1371\/journal.pone.0095519","article-title":"Mannose binding lectin and susceptibility to rheumatoid arthritis in Brazilian patients and their relatives","volume":"9","author":"Goeldner","year":"2014","journal-title":"PLoS One"},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib231","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1002\/1529-0131(200003)43:3<515::AID-ANR6>3.0.CO;2-T","article-title":"The association of variant mannose-binding lectin genotypes with radiographic outcome in rheumatoid arthritis","volume":"43","author":"Graudal","year":"2000","journal-title":"Arthritis Rheum."},{"issue":"1","key":"10.1016\/j.jaut.2022.102979_bib232","first-page":"26","article-title":"Two edged role of mannose binding lectin in rheumatoid arthritis: a cross sectional study","volume":"27","author":"Garred","year":"2000","journal-title":"J. Rheumatol."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib233","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1159\/000019099","article-title":"An unequal crossover between the RCCX modules of the human MHC leading to the presence of a CYP21B gene and a tenascin TNXB\/TNXA-RP2 recombinant between C4A and C4B genes in a patient with juvenile rheumatoid arthritis","volume":"16","author":"Rupert","year":"1999","journal-title":"Exp. Clin. Immunogenet."},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib234","doi-asserted-by":"crossref","first-page":"3815","DOI":"10.2337\/dc13-0203","article-title":"Increased complement activation in human type 1 diabetes pancreata","volume":"36","author":"Rowe","year":"2013","journal-title":"Diabetes Care"},{"issue":"5","key":"10.1016\/j.jaut.2022.102979_bib235","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1111\/j.1399-5448.2011.00836.x","article-title":"Gene CNVs and protein levels of complement C4A and C4B as novel biomarkers for partial disease remissions in new-onset type 1 diabetes patients","volume":"13","author":"Kingery","year":"2012","journal-title":"Pediatr. Diabetes"},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib236","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1093\/hmg\/10.8.881","article-title":"Conditional linkage disequilibrium analysis of a complex disease superlocus, IDDM1 in the HLA region, reveals the presence of independent modifying gene effects influencing the type 1 diabetes risk encoded by the major HLA-DQB1, -DRB1 disease loci","volume":"10","author":"Zavattari","year":"2001","journal-title":"Hum. Mol. Genet."},{"issue":"7171","key":"10.1016\/j.jaut.2022.102979_bib237","doi-asserted-by":"crossref","first-page":"887","DOI":"10.1038\/nature06406","article-title":"Localization of type 1 diabetes susceptibility to the MHC class I genes HLA-B and HLA-A","volume":"450","author":"Nejentsev","year":"2007","journal-title":"Nature"},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib238","doi-asserted-by":"crossref","first-page":"2972","DOI":"10.2337\/db10-0699","article-title":"HLA class I and genetic susceptibility to type 1 diabetes: results from the Type 1 Diabetes Genetics Consortium","volume":"59","author":"Noble","year":"2010","journal-title":"Diabetes"},{"key":"10.1016\/j.jaut.2022.102979_bib239","doi-asserted-by":"crossref","first-page":"27887","DOI":"10.1038\/srep27887","article-title":"Complement gene variants in relation to autoantibodies to beta cell specific antigens and type 1 diabetes in the TEDDY Study","volume":"6","author":"T\u00f6rn","year":"2016","journal-title":"Sci. Rep."},{"issue":"6","key":"10.1016\/j.jaut.2022.102979_bib240","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1080\/14397595.2021.1886627","article-title":"Lupus manifestations in children with primary immunodeficiency diseases: comprehensive phenotypic and genetic features and outcome","volume":"31","author":"Al-Mayouf","year":"2021","journal-title":"Mod. Rheumatol."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib241","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1007\/s00439-018-01966-7","article-title":"Whole-genome sequencing identifies complex contributions to genetic risk by variants in genes causing monogenic systemic lupus erythematosus","volume":"138","author":"Alml\u00f6f","year":"2019","journal-title":"Hum. Genet."},{"issue":"3","key":"10.1016\/j.jaut.2022.102979_bib242","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1016\/j.ajhg.2012.01.012","article-title":"Genetically determined partial complement C4 deficiency states are not independent risk factors for SLE in UK and Spanish populations","volume":"90","author":"Boteva","year":"2012","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.jaut.2022.102979_bib243","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1186\/1423-0127-18-71","article-title":"Association between copy number variation of complement component C4 and Graves' disease","volume":"18","author":"Liu","year":"2011","journal-title":"J. Biomed. Sci."},{"issue":"8","key":"10.1016\/j.jaut.2022.102979_bib244","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1038\/sj.gene.6363804","article-title":"Association of mannose-binding lectin gene variation with disease severity and infections in a population-based cohort of systemic lupus erythematosus patients","volume":"2","author":"Garred","year":"2001","journal-title":"Gene Immun."},{"key":"10.1016\/j.jaut.2022.102979_bib245","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1016\/j.molimm.2017.01.015","article-title":"Potential influences of complement factor H in autoimmune inflammatory and thrombotic disorders","volume":"84","author":"Ferluga","year":"2017","journal-title":"Mol. Immunol."},{"issue":"2","key":"10.1016\/j.jaut.2022.102979_bib246","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1038\/sj.gene.6363744","article-title":"Mannose-binding lectin gene: polymorphisms in Japanese patients with systemic lupus erythematosus, rheumatoid arthritis and Sj\u00f6gren's syndrome","volume":"2","author":"Tsutsumi","year":"2001","journal-title":"Gene Immun."},{"key":"10.1016\/j.jaut.2022.102979_bib247","doi-asserted-by":"crossref","DOI":"10.1002\/art.42122","article-title":"Complement C4 copy number variation is linked to SSA\/Ro and SSB\/La autoantibodies in systemic inflammatory autoimmune diseases","author":"Lundtoft","year":"2022","journal-title":"Arthritis Rheumatol."},{"issue":"7813","key":"10.1016\/j.jaut.2022.102979_bib248","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1038\/s41586-020-2277-x","volume":"582","author":"Kamitaki","year":"2020","journal-title":"Nature"},{"key":"10.1016\/j.jaut.2022.102979_bib249","series-title":"Systemic Lupus Erythematosus","first-page":"21","article-title":"Genetic susceptibility and class III complement genes","author":"Atkinson","year":"2011"},{"issue":"11","key":"10.1016\/j.jaut.2022.102979_bib250","doi-asserted-by":"crossref","first-page":"8466","DOI":"10.1016\/S0021-9258(17)37217-4","article-title":"Structure and genetics of the partially duplicated gene RP located immediately upstream of the complement C4A and the C4B genes in the HLA class III region. Molecular cloning, exon-intron structure, composite retroposon, and breakpoint of gene duplication","volume":"269","author":"Shen","year":"1994","journal-title":"J. Biol. Chem."}],"container-title":["Journal of Autoimmunity"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0896841122001871?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0896841122001871?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T08:31:30Z","timestamp":1760257890000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0896841122001871"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5]]},"references-count":250,"alternative-id":["S0896841122001871"],"URL":"https:\/\/doi.org\/10.1016\/j.jaut.2022.102979","relation":{},"ISSN":["0896-8411"],"issn-type":[{"value":"0896-8411","type":"print"}],"subject":[],"published":{"date-parts":[[2023,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"The complement system and human autoimmune diseases","name":"articletitle","label":"Article Title"},{"value":"Journal of Autoimmunity","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jaut.2022.102979","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2022 The Authors. Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}],"article-number":"102979"}}