{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T05:52:32Z","timestamp":1790488352257,"version":"4.1.0"},"reference-count":35,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2000,5,1]],"date-time":"2000-05-01T00:00:00Z","timestamp":957139200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2001,12,24]]},"abstract":"<jats:title>SUMMARY<\/jats:title><jats:p>Individuals with either a late (C5\u20139) complement component deficiency (LCCD) or properdin deficiency are at increased risk to develop meningococcal disease, often due to serogroups W135 and Y. Anti-meningococcal defence in both LCCD persons and properdin-deficient individuals without bactericidal antibodies depends mainly on phagocytosis. Three types of opsonin receptors are involved in phagocytosis by polymorphonuclear cells (PMN). These represent the polymorphic Fc\u03b3RIIa (CD32) and Fc\u03b3RIIIb (CD16b) receptors, and the C3 receptor CR3 (CD11b\/CD18). When the distribution of Fc\u03b3RIIa and Fc\u03b3RIIIb allotypes was assessed in 15 LCCD and in 15 properdin-deficient patients with\/without previous meningococcal disease, we found the combination of Fc\u03b3RIIa-R\/R131 with Fc\u03b3RIIIb-NA2\/NA2 allotypes to be associated with previous meningococcal disease (odds ratio 13\u00b79, Fisher\u2019s test P = 0\u00b7036). No such relation was observed in the properdin-deficient patients. The importance of Fc\u03b3RIIa allotypes was also demonstrated using in\u2003vitro phagocytosis assays. PMN from Fc\u03b3RIIa-R\/R131 homozygous donors internalized IgG2 opsonized meningococci W135 significantly (P\u2003&amp;lt;\u20030\u00b705) less than PMN from Fc\u03b3RIIa-H\/H131 donors. When properdin-deficient serum was tested, it was observed that reconstitution with properdin resulted in enhanced PMN phagocytosis of the W135 meningococci (P = 0\u00b7001). This enhanced phagocytosis was parallelled by an increase in C3 deposition onto the opsonized meningococci W135 (r = 0\u00b76568, P = 0\u00b701). We conclude that the occurrence of meningococcal disease in LCCD patients is associated with certain Fc\u03b3R allotypes. Properdin-deficient individuals are susceptible to meningococcal disease because of an insufficient C3 deposition on the surface of meningococci, resulting in insufficient phagocytosis.<\/jats:p>","DOI":"10.1046\/j.1365-2249.2000.01208.x","type":"journal-article","created":{"date-parts":[[2003,3,11]],"date-time":"2003-03-11T10:21:27Z","timestamp":1047378087000},"page":"338-345","source":"Crossref","is-referenced-by-count":67,"title":["The role of Fc<i>\u03b3<\/i>receptor polymorphisms and C3 in the immune defence against<i>Neisseria meningitidis<\/i>in complement-deficient individuals"],"prefix":"10.1093","volume":"120","author":[{"given":"C A P","family":"Fijen","sequence":"first","affiliation":[{"name":"Department of Medical Microbiology, Reference Laboratory for Bacterial Meningitis, University of Amsterdam, Amsterdam and National Institute for Public Health and Environmental Protection, Bilthoven"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R G M","family":"Bredius","sequence":"additional","affiliation":[{"name":"Clinical Laboratory and Immunology Unit, Emma Children\u2019s Hospital and Department of Paediatrics, Academic Medical Centre and"},{"name":"Clinical Immunology (CLB, Sanquin Blood Supply Foundation), University of Amsterdam, Amsterdam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E J","family":"Kuijper","sequence":"additional","affiliation":[{"name":"Department of Medical Microbiology, Reference Laboratory for Bacterial Meningitis, University of Amsterdam, Amsterdam and National Institute for Public Health and Environmental Protection, Bilthoven"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T A","family":"Out","sequence":"additional","affiliation":[{"name":"Clinical Laboratory and Immunology Unit, Emma Children\u2019s Hospital and Department of Paediatrics, Academic Medical Centre and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"De Haas","sequence":"additional","affiliation":[{"name":"Clinical Immunology (CLB, Sanquin Blood Supply Foundation), University of Amsterdam, Amsterdam"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A P M","family":"De Wit","sequence":"additional","affiliation":[{"name":"Department of Immunology and Medarex Europe, University Medical Centre Utrecht, Utrecht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M R","family":"Daha","sequence":"additional","affiliation":[{"name":"Department of Nephrology, University Hospital Leiden, Leiden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J G J","family":"Van De Winkel","sequence":"additional","affiliation":[{"name":"Department of Immunology and Medarex Europe, University Medical Centre Utrecht, Utrecht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2001,12,24]]},"reference":[{"key":"2022011221075023800_b1","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1128\/CMR.4.3.359","article-title":"Infectious disease associated with complement deficiencies","volume":"4","author":"Figueroa","year":"1991","journal-title":"Clin Microbiol Rev"},{"key":"2022011221075023800_b2","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1016\/S0140-6736(89)90712-5","article-title":"Complement deficiencies in patients over ten years old with meningococcal disease due to uncommon serogroups","volume":"ii","author":"Fijen","year":"1989","journal-title":"Lancet"},{"key":"2022011221075023800_b3","doi-asserted-by":"crossref","first-page":"2185","DOI":"10.1128\/jcm.32.9.2185-2191.1994","article-title":"Characterization of strains of Neisseria meningitidis recovered from complement-normal and complement-deficient patients in the Cape, South Africa","volume":"32","author":"Orren","year":"1994","journal-title":"J Clin Microbiol"},{"key":"2022011221075023800_b4","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1084\/jem.129.6.1307","article-title":"Human immunity to the meningococcus. 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