{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T04:40:09Z","timestamp":1792644009042,"version":"4.5.0"},"reference-count":24,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JASN"],"published-print":{"date-parts":[[2017,5]]},"abstract":"<jats:p>\n                    In C3 glomerulopathy (C3G), the alternative pathway of complement is frequently overactivated by autoantibodies that stabilize the C3 convertase C3bBb. Anti-C3b and anti-factor B (anti-FB) IgG have been reported in three patients with C3G. We screened a cohort of 141 patients with C3G and Ig-associated membranoproliferative GN (Ig-MPGN) for anti-FB and anti-C3b autoantibodies using ELISA. We identified seven patients with anti-FB IgG, three patients with anti-C3b IgG, and five patients with anti-FB and anti-C3b IgG. Of these 15 patients, ten were diagnosed with Ig-MPGN. Among those patients with available data, 92% had a nephrotic syndrome, 64% had AKI, and 67% had a documented infection. Patients negative for anti-C3b and anti-FB IgG had much lower rates of infection (17 [25%] patients with C3G and one [10%] patient with Ig-MPGN). After 48 months, four of 15 (26%) positive patients had developed ESRD or died. All 15 patients had high plasma Bb levels, six (40%) patients had low levels of C3, and nine (60%) patients had high levels of soluble C5b9.\n                    <jats:italic toggle=\"yes\">In vitro<\/jats:italic>\n                    , IgG purified from patients with anti-FB Abs selectively enhanced C3 convertase activity; IgG from patients with anti-C3b\/anti-FB Abs enhanced C3 and C5 cleavage. IgG from patients with anti-C3b Abs stabilized C3bBb and perturbed C3b binding to complement receptor 1 but did not perturb binding to factor H. In conclusion, the prevalence of anti-C3b\/anti-FB Abs and alternative pathway activation is similar in Ig-MPGN and C3G, suggesting similar pathogenic mechanisms. Identification of the underlying defect in Ig-MPGN could lead to improved treatment.\n                  <\/jats:p>","DOI":"10.1681\/asn.2016030343","type":"journal-article","created":{"date-parts":[[2017,1,17]],"date-time":"2017-01-17T21:10:46Z","timestamp":1484687446000},"page":"1603-1613","source":"Crossref","is-referenced-by-count":114,"title":["Anti-Factor B and Anti-C3b Autoantibodies in C3 Glomerulopathy and Ig-Associated Membranoproliferative GN"],"prefix":"10.1681","volume":"28","author":[{"given":"Maria Chiara","family":"Marinozzi","sequence":"first","affiliation":[{"name":"Team Complement and Diseases Cordeliers Research Center, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Unit\u00e9 Mixte de Recherche 1138, Paris, France;"},{"name":"Assistance Publique-H\u00f4pitaux de Paris, Service d\u2019Immunologie Biologique, H\u00f4pital Europ\u00e9en Georges Pompidou, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lubka T.","family":"Roumenina","sequence":"additional","affiliation":[{"name":"Team Complement and Diseases Cordeliers Research Center, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Unit\u00e9 Mixte de Recherche 1138, Paris, France;"},{"name":"Universit\u00e9 Paris Descartes Sorbonne Paris-Cit\u00e9, Paris, France;"},{"name":"Universit\u00e9 Pierre et Marie Curie, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Chauvet","sequence":"additional","affiliation":[{"name":"Team Complement and Diseases Cordeliers Research Center, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Unit\u00e9 Mixte de Recherche 1138, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre","family":"Hertig","sequence":"additional","affiliation":[{"name":"Assistance Publique-H\u00f4pitaux de Paris, H\u00f4pital Tenon, Service de N\u00e9phrologie et de Transplantation r\u00e9nale, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Bertrand","sequence":"additional","affiliation":[{"name":"Service de N\u00e9phrologie et de Transplantation r\u00e9nale, H\u00f4pital Bois Guillaume, Rouen, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00e9rome","family":"Olagne","sequence":"additional","affiliation":[{"name":"Service de N\u00e9phrologie et de Transplantation r\u00e9nale, Strasbourg, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Frimat","sequence":"additional","affiliation":[{"name":"Service de N\u00e9phrologie et de Transplantation r\u00e9nale, Lille, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tim","family":"Ulinski","sequence":"additional","affiliation":[{"name":"Assistance Publique-H\u00f4pitaux de Paris, Service de N\u00e9phrologie, H\u00f4pital Trousseau, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georges","family":"Desch\u00eanes","sequence":"additional","affiliation":[{"name":"Assistance Publique-H\u00f4pitaux de Paris, Service de N\u00e9phrologie et de Transplantation r\u00e9nale, H\u00f4pital Robert Debr\u00e9, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephane","family":"Burtey","sequence":"additional","affiliation":[{"name":"Aix Marseille universit\u00e9, Assistance publique-H\u00f4pitaux de Marseille, Service de N\u00e9phrologie et de Transplantation r\u00e9nale, Marseille, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Delahousse","sequence":"additional","affiliation":[{"name":"Service de N\u00e9phrologie et Transplantation r\u00e9nale, Hopital Foch, Paris, France; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Moulin","sequence":"additional","affiliation":[{"name":"Service de N\u00e9phrologie et de Transplantation r\u00e9nale, Strasbourg, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christophe","family":"Legendre","sequence":"additional","affiliation":[{"name":"Assistance Publique-H\u00f4pitaux de Paris, Service de N\u00e9phrologie et de Transplantation r\u00e9nale, H\u00f4pital Necker, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V\u00e9ronique","family":"Fr\u00e9meaux-Bacchi","sequence":"additional","affiliation":[{"name":"Team Complement and Diseases Cordeliers Research Center, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Unit\u00e9 Mixte de Recherche 1138, Paris, France;"},{"name":"Assistance Publique-H\u00f4pitaux de Paris, Service d\u2019Immunologie Biologique, H\u00f4pital Europ\u00e9en Georges Pompidou, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moglie","family":"Le Quintrec","sequence":"additional","affiliation":[{"name":"Team 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