{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T07:11:47Z","timestamp":1767165107440,"version":"3.48.0"},"reference-count":32,"publisher":"The Royal Society","issue":"1129","license":[{"start":{"date-parts":[[1984,9,6]],"date-time":"1984-09-06T00:00:00Z","timestamp":463276800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/royalsociety.org\/journals\/ethics-policies\/data-sharing-mining\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1984,9,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Isolated genetic deficiencies of individual components of the complement system have been described in man for all the components of the classical pathway and the membrane attack complex as well as for Factor I, Factor H and properdin. It is only for Factor B and Factor D of the alternative pathway that homozygous deficiency states are not so far known. Complement deficiency states provide the most direct way of looking at the role of the complement system in vivo and emphasize the importance of complement in resistance to bacterial infection and in particular to infection with Neisseria. This association is not unexpected since in vitro studies have shown complement to be an efficient enhancer of phagocytosis and inflammation. The particularly frequent occurrence of neisserial infection may be ascribed to the ability of these organisms to survive in phagocytic cells so that the plasma cytolytic activity provided by complement is needed to kill them. On the other hand the strong association between complement deficiencies and immune-complex diseases - especially systemic lupus erythematosus \u2014 was unexpected and seems paradoxical in view of the large part played by complement in the pathogenesis of immune complex mediated tissue damage. The paradox can be explained in part by the necessity for an intact complement system in the solubilization and the proper handling of immune complexes. It is also likely that complement deficiency can allow the persistence of low virulence organisms that produce disease solely by an immune complex mechanism. Recently described deficiencies of complement receptors and their effects in vivo are described.<\/jats:p>","DOI":"10.1098\/rstb.1984.0102","type":"journal-article","created":{"date-parts":[[2006,12,15]],"date-time":"2006-12-15T16:39:30Z","timestamp":1166200770000},"page":"419-430","source":"Crossref","is-referenced-by-count":20,"title":["Inherited complement deficiencies"],"prefix":"10.1098","volume":"306","author":[{"given":"Peter Julius","family":"Lachmann","sequence":"first","affiliation":[{"name":"Medical Research Council Centre, University Medical School, Hills Road, Cambridge CB2 2QH, U. K."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"175","reference":[{"key":"p_1","first-page":"693","article-title":"Deficiency of a granulocyte-membrane glycoprotein (gpl50) in a boy with recurrent bacterial infections. New Engl. J","volume":"306","author":"Arnaout M. 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Immunobiology"},{"key":"p_8","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2222.1971.tb03021.x"},{"key":"p_9","first-page":"542","article-title":"Essentielle","volume":"27","author":"Hassig A.","year":"1964","journal-title":"Hypokomplementamie. Pathol. Microbiol."},{"key":"p_10","unstructured":"Hauptmann G. Goetz J. Uring-Lambert B. & Grosshans E. 1984 C4 deficiency. Progr. Allergy (In the press.)"},{"key":"p_11","unstructured":"Inai S. & Akagaki Y. 1984 The incidence of C9 deficiency in Japan. Proceedings of the International Symposium on Frontiers of Complement Research. Prog. Immunol. 5 (In the press.)"},{"key":"p_12","doi-asserted-by":"publisher","DOI":"10.1016\/S0065-2776(08)60527-1"},{"key":"p_13","unstructured":"Lachmann P. J. 1984 Deficiencies of Factor I and H. In The complement system (ed. K. Rother and G. Till). New York: Springer-Verlag (In preparation.)"},{"key":"p_14","first-page":"193","article-title":"Combined genetic deficiency of C6 and C7 in man. Clin, exp","volume":"33","author":"Lachmann P. J.","year":"1978","journal-title":"Immunol."},{"key":"p_15","doi-asserted-by":"publisher","DOI":"10.1016\/0161-5890(82)90098-0"},{"key":"p_16","unstructured":"Martin H. McConnell I. Gorick B. & Hughes-Jones N. C. 1984 Antibody-independent activation of the classical pathway of complement by Epstein-Barr virus. (In preparation.)"},{"key":"p_17","first-page":"331","article-title":"Prevalence of deficits of complement components in patients with recurrent meningococcal infections. J. infect","volume":"148","author":"Merino J.","year":"1983","journal-title":"Dis."},{"key":"p_18","doi-asserted-by":"crossref","unstructured":"Mevag B. Rogde S. Olaisen B. Gedde-Dahl T. Teisberg P. & Tedesco F. 1984 Genetic polymorphism of complement component C8. Proceedings Xth Congress of Forensic Haemogenetics Munich 1983.","DOI":"10.1016\/0379-0738(83)90174-3"},{"key":"p_19","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.72.2.418"},{"key":"p_20","first-page":"483","article-title":"Pathogenesis of chronic disease associated with persistent Iymphocytic choriomeningitis viral infection. I. Relationship of antibody production to disease in neonatally infected mice. J. exp","volume":"129","author":"Oldstone M. B. A.","year":"1969","journal-title":"Med."},{"key":"p_21","doi-asserted-by":"publisher","DOI":"10.1172\/JCI108279"},{"key":"p_22","unstructured":"Ross G. D. Thompson R. A. Walport M. J. Ward R. H. R. Lida J. Newman S. L. Harrison R. A. & Lachmann P. J. 1984a Identification of a genetic deficiency of leukocyte membrane complement receptor type three (CR3 an iC3b receptor) and its association with increased susceptibility to bacterial infections. (In the press.)"},{"key":"p_23","unstructured":"Ross G. D. Walport M. J. & Lachmann P. J. 1984^ (In preparation.)"},{"key":"p_24","doi-asserted-by":"crossref","unstructured":"Schifferli J. A. & Peters D. K. 1983 Complement the immune-complex lattice and the pathophysiology of complement-deficiency syndromes. Lancet i 957.","DOI":"10.1016\/S0140-6736(83)90464-6"},{"key":"p_25","first-page":"11997","article-title":"The eighth component of human complement: purification and physicochemical characterization of its unusual subunit structure. J. biol","volume":"255","author":"Steckel E. W.","year":"1980","journal-title":"Chem."},{"key":"p_26","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1016\/S0260-4639(22)00027-5","article-title":"Complement-dependent solubilisation of immune complexes","volume":"1","author":"Takahashi M.","year":"1981","journal-title":"Clin. Immunol. 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