Abstract
The serum of a 23-yr-old woman with prolonged disseminated gonococcal infection syndrome failed to normally promote hemolysis of sensitized sheep red blood cells (RBC). The patient's serum was deficient in the eight component of complement (C8) as determined by functional assays, immunoelectrophoresis, and quantitative immunoprecipitation. Functional titers of each of her other complement components were normal. No serum inhibitors of C8 were detected. The patient's serum supported activation of both the classical and alternate complement pathways. Her fresh serum lacked any bactericidal activity against Neisseria gonorrhoeae, but addition of purified C8 or complement donor serum restored bactericidal activity as well as RBC hemolytic activity. Her serum gave normal opsonization of yeast particles and staphylococci and had normal capacity to coat sensitized RBC with C8 and C4 and to generate chemotactic activity. No defects were observed in the patient's blood coagulation mechanisms. Complement-mediated bacterial lysis may be important in human defense against bacteremic Neisseria infections.
Full text
PDF







Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Alper C. A., Abramson N., Johnston R. B., Jr, Jandl J. H., Rosen F. S. Increased susceptibility to infection associated with abnormalities of complement-mediated functions and of the third component of complement (C3). N Engl J Med. 1970 Feb 12;282(7):350–354. doi: 10.1056/nejm197002122820701. [DOI] [PubMed] [Google Scholar]
- Alper C. A., Bloch K. J., Rosen F. S. Increased susceptibility to infection in a patient with type II essential hypercatabolism of C3. N Engl J Med. 1973 Mar 22;288(12):601–606. doi: 10.1056/NEJM197303222881204. [DOI] [PubMed] [Google Scholar]
- Alper C. A., Colten H. R., Rosen F. S., Rabson A. R., Macnab G. M., Gear J. S. Homozygous deficiency of C3 in a patient with repeated infections. Lancet. 1972 Dec 2;2(7788):1179–1181. doi: 10.1016/s0140-6736(72)92598-6. [DOI] [PubMed] [Google Scholar]
- Boyer J. T., Gall E. P., Norman M. E., Nilsson U. R., Zimmerman T. S. Hereditary deficiency of the seventh component of complement. J Clin Invest. 1975 Oct;56(4):905–913. doi: 10.1172/JCI108170. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Böyum A. Isolation of mononuclear cells and granulocytes from human blood. Isolation of monuclear cells by one centrifugation, and of granulocytes by combining centrifugation and sedimentation at 1 g. Scand J Clin Lab Invest Suppl. 1968;97:77–89. [PubMed] [Google Scholar]
- Colman R. W., Mattler L., Sherry S. Studies on the prekallikrein (kallikreinogen)--kallikrein enzyme system of human plasma. II. Evidence relating the kaolin-activated arginine esterase to plasma kallikrein. J Clin Invest. 1969 Jan;48(1):23–32. doi: 10.1172/JCI105971. [DOI] [PMC free article] [PubMed] [Google Scholar]
- DONALDSON V. H., EVANS R. R. A BIOCHEMICAL ABNORMALITY IN HEREDIATRY ANGIONEUROTIC EDEMA: ABSENCE OF SERUM INHIBITOR OF C' 1-ESTERASE. Am J Med. 1963 Jul;35:37–44. doi: 10.1016/0002-9343(63)90162-1. [DOI] [PubMed] [Google Scholar]
- Ensky J., Hinz C. F., Jr, Todd E. W., Wedgwood R. J., Boyer J. T., Lepow I. H. Properties of highly purified human properdin. J Immunol. 1968 Jan;100(1):142–158. [PubMed] [Google Scholar]
- Fass R. J., Saslaw S. Chronic meningococcemia. Possible pathogenic role of IgM deficiency. Arch Intern Med. 1972 Dec;130(6):943–946. doi: 10.1001/archinte.130.6.943. [DOI] [PubMed] [Google Scholar]
- Gibbs D. L., Roberts R. B. The interaction in vitro between human polymorphonuclear leukocytes and Neisseria gonorrhoeae cultivated in the chick embryo. J Exp Med. 1975 Jan 1;141(1):155–171. doi: 10.1084/jem.141.1.155. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Gigli I., Nelson R. A., Jr Complement dependent immune phagocytosis. I. Requirements for C'1, C'4, C'2, C'3. Exp Cell Res. 1968 Jul;51(1):45–67. doi: 10.1016/0014-4827(68)90158-4. [DOI] [PubMed] [Google Scholar]
- Goldschneider I., Gotschlich E. C., Artenstein M. S. Human immunity to the meningococcus. I. The role of humoral antibodies. J Exp Med. 1969 Jun 1;129(6):1307–1326. doi: 10.1084/jem.129.6.1307. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Götze O., Müller-Eberhard H. J. The C3-activator system: an alternate pathway of complement activation. J Exp Med. 1971 Sep 1;134(3 Pt 2):90s–108s. [PubMed] [Google Scholar]
- Hattersley P. G. Activated coagulation time of whole blood. JAMA. 1966 May 2;196(5):436–440. [PubMed] [Google Scholar]
- Holmes K. K., Counts G. W., Beaty H. N. Disseminated gonococcal infection. Ann Intern Med. 1971 Jun;74(6):979–993. doi: 10.7326/0003-4819-74-6-979. [DOI] [PubMed] [Google Scholar]
- Horwitz D. A., Garrett M. A. Use of leukocyte chemotaxis in vitro to assay mediators generated by immune reactions. I. Quantitation of mononuclear and polymorphonuclear leukocyte chemotaxis with polycarbonate (nuclepore) filters. J Immunol. 1971 Mar;106(3):649–655. [PubMed] [Google Scholar]
- Jasin H. E. Human heat labile opsonins: evidence for their mediation via the alternate pathway of complement activation. J Immunol. 1972 Jul;109(1):26–31. [PubMed] [Google Scholar]
- KELLOGG D. S., Jr, PEACOCK W. L., Jr, DEACON W. E., BROWN L., PIRKLE D. I. NEISSERIA GONORRHOEAE. I. VIRULENCE GENETICALLY LINKED TO CLONAL VARIATION. J Bacteriol. 1963 Jun;85:1274–1279. doi: 10.1128/jb.85.6.1274-1279.1963. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Klebanoff S. J., Hamon C. B. Role of myeloperoxidase-mediated antimicrobial systems in intact leukocytes. J Reticuloendothel Soc. 1972 Aug;12(2):170–196. [PubMed] [Google Scholar]
- Leddy J. P., Frank M. M., Gaither T., Baum J., Klemperer M. R. Hereditary deficiency of the sixth component of complement in man. I. Immunochemical, biologic, and family studies. J Clin Invest. 1974 Feb;53(2):544–553. doi: 10.1172/JCI107588. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Mancini G., Carbonara A. O., Heremans J. F. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235–254. doi: 10.1016/0019-2791(65)90004-2. [DOI] [PubMed] [Google Scholar]
- Manni J. A., Müller-Eberhard H. J. The eighth component of human complement (C8): isolation, characterization, and hemolytic efficiency. J Exp Med. 1969 Nov 1;130(5):1145–1160. doi: 10.1084/jem.130.5.1145. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Miller M. E., Nilsson U. R. A familial deficiency of the phagocytosis-enhancing activity of serum related to a dysfunction of the fifth component of complement (C5). N Engl J Med. 1970 Feb 12;282(7):354–358. doi: 10.1056/NEJM197002122820702. [DOI] [PubMed] [Google Scholar]
- Nelson R. A., Jr, Jensen J., Gigli I., Tamura N. Methods for the separation, purification and measurement of nine components of hemolytic complement in guinea-pig serum. Immunochemistry. 1966 Mar;3(2):111–135. doi: 10.1016/0019-2791(66)90292-8. [DOI] [PubMed] [Google Scholar]
- Pincus S. H., Klebanoff S. J. Quantitative leukocyte iodination. N Engl J Med. 1971 Apr 8;284(14):744–750. doi: 10.1056/NEJM197104082841402. [DOI] [PubMed] [Google Scholar]
- Polley M. J., Bearn A. G. Genetic aspects of diseases of complement: an explosion. Am J Med. 1975 Jan;58(1):105–111. doi: 10.1016/0002-9343(75)90540-9. [DOI] [PubMed] [Google Scholar]
- Punsalang A. P., Jr, Sawyer W. D. Role of pili in the virulence of Neisseria gonorrhoeae. Infect Immun. 1973 Aug;8(2):255–263. doi: 10.1128/iai.8.2.255-263.1973. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Root R. K., Ellman L., Frank M. M. Bactericidal and opsonic properties of C4-deficient guinea pig serum. J Immunol. 1972 Sep;109(3):477–486. [PubMed] [Google Scholar]
- Ruddy S., Gigli I., Austen K. F. The complement system of man. I. N Engl J Med. 1972 Sep 7;287(10):489–495. doi: 10.1056/NEJM197209072871005. [DOI] [PubMed] [Google Scholar]
- SCHEIDEGGER J. J. Une micro-méthode de l'immuno-electrophorèse. Int Arch Allergy Appl Immunol. 1955;7(2):103–110. [PubMed] [Google Scholar]
- Swanson J., Sparks E., Zeligs B., Siam M. A., Parrott C. Studies on gonococcus infection. V. Observations on in vitro interactions of gonococci and human neutrophils. Infect Immun. 1974 Sep;10(3):633–644. doi: 10.1128/iai.10.3.633-644.1974. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Swanson J., Zeligs B. Studies on gonococcus infection. VI. Electron microscopic study on in vitro phagocytosis of gonococci by human leukocytes. Infect Immun. 1974 Sep;10(3):645–656. doi: 10.1128/iai.10.3.645-656.1974. [DOI] [PMC free article] [PubMed] [Google Scholar]
- TALIAFERRO L. G., TALIAFERRO W. H. X-ray effects on hemolysin formation in rabbits with the spleen shielded or irradiated. J Infect Dis. 1956 Sep-Oct;99(2):109–128. doi: 10.1093/infdis/99.2.109. [DOI] [PubMed] [Google Scholar]
- Thongthai C., Sawyer W. D. Studies on the virulence of Neisseria gonorrhoeae. I. Relation of colonial morphology and resistance to phagocytosis by polymorphonuclear leukocytes. Infect Immun. 1973 Mar;7(3):373–379. doi: 10.1128/iai.7.3.373-379.1973. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Wellek B., Opferkuch W. A case of deficiency of the seventh component of complement in man. Biological properties of a C7-deficient serum and description of a C7-inactivating principle. Clin Exp Immunol. 1975 Feb;19(2):223–235. [PMC free article] [PubMed] [Google Scholar]