{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T20:33:29Z","timestamp":1775075609651,"version":"3.50.1"},"reference-count":20,"publisher":"Rockefeller University Press","issue":"11","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2001,12,3]]},"abstract":"<jats:p>We studied complement 1 inhibitor (C1-INH) as an inhibitor of the alternative complement pathway. C1-INH prevented lysis, induced by the alternative complement pathway, of paroxysmal nocturnal hemoglobinuria (PNH) erythrocytes in human serum. It inhibited the binding of both factors B and C3 to PNH and rabbit erythrocytes and blocked the ability of factor B to restore alternative-pathway function in factor B\u2013depleted serum. C1-INH did not bind to factors B or D but did bind to immobilized C3b and cobra venom factor (CVF), a C3b analogue. C1-INH prevented factor B from binding to CVF-coated beads and dissociated bound factor B from such beads. Factor B and C1-INH showed cross competition in binding to CVF-coated beads. Factor D cleaved factor B into Bb and Ba in the presence of C3b. Cleavage was markedly inhibited when C3b was preincubated with C1-INH. C1-INH inhibited the formation of CVFBb and decreased the C3 cleavage. Removal of C1-INH from serum, in the presence of Mg-EGTA with an anti\u2013C1-INH immunoabsorbant, markedly increased alternative-pathway lysis. C1-INH interacts with C3b to inhibit binding of factor B to C3b. At physiologic concentrations, it is a downregulator of the alternative pathway convertase.<\/jats:p>","DOI":"10.1084\/jem.194.11.1609","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T16:48:33Z","timestamp":1027702113000},"page":"1609-1616","update-policy":"https:\/\/doi.org\/10.1084\/jem.crossmarkpolicy","source":"Crossref","is-referenced-by-count":123,"title":["Complement 1 Inhibitor Is a Regulator of the Alternative Complement Pathway"],"prefix":"10.1084","volume":"194","author":[{"given":"Haixiang","family":"Jiang","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Duke University Medical Center, Durham, NC 27710"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eric","family":"Wagner","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Duke University Medical Center, Durham, NC 27710"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huamei","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Duke University Medical Center, Durham, NC 27710"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael M.","family":"Frank","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Duke University Medical Center, Durham, NC 27710"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"291","published-online":{"date-parts":[[2001,12,3]]},"reference":[{"key":"2023072509474427100_BIB1"},{"key":"2023072509474427100_BIB2","doi-asserted-by":"crossref","first-page":"608","DOI":"10.3181\/00379727-101-25034","volume":"101","year":"1959","journal-title":"Proc. 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