Inherited complement deficiencies

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Abstract

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 — 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.

Publisher

The Royal Society

Subject

Industrial and Manufacturing Engineering,General Agricultural and Biological Sciences,General Business, Management and Accounting,Materials Science (miscellaneous),Business and International Management

Cited by 28 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Prédisposition génétique aux maladies infectieuses chez l’homme;Immuno-analyse & Biologie Spécialisée;2013-08

2. Complement Factor D;Handbook of Proteolytic Enzymes;2013

3. Autoimmunity: Homeostasis of Innate Immunity Gone Awry;Journal of Clinical Immunology;2012-10-11

4. Acetohydroxamate, a Urease Inhibitor, Inhibits the Covalent Binding Reaction of Complement Proteins C3 and C4;Acta Pharmacologica et Toxicologica;2009-03-13

5. Deficiency of the Effector Mechanisms of the Immune Response and Autoimmunity;Ciba Foundation Symposium 129 - Autoimmunity and Autoimmune Disease;2007-09-28

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