Synonyms
Innate immune system
Definition
The complement system is a family of liver-derived, circulating proteins and represents an important and central humoral part of innate immunity that is well organized as a complex network comprising inactive protein precursors, fluid-phase and membrane-bound regulators and inhibitors, as well as newly generated peptide cleavage products with multiple effector functions (Walport 2001a, b; Zipfel 2009; Ricklin et al. 2010; Trouw and Daha 2011). As a first line of defense, this powerful immune surveillance system plays a substantial role in the discrimination between “self” and “nonself,” a prerequisite for recognition and elimination of intruding microorganisms; for the clearance of necrotic and apoptotic cells, circulating immune complexes, and cellular debris; as well as for the promotion of tissue regeneration and, thus, supports tissue homeostasis. If not properly controlled or under imbalanced physiological conditions, complement...
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References
Amara, U., Rittirsch, D., Flierl, M., Bruckner, U., Klos, A., Gebhard, F., et al. (2008). Interaction between the coagulation and complement system. [Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov’t Review]. Advances in Experimental Medicine and Biology, 632, 71–79.
Appel, G. B., Cook, H. T., Hageman, G., Jennette, J. C., Kashgarian, M., Kirschfink, M., et al. (2005). Membranoproliferative glomerulonephritis type II (dense deposit disease): An update. [Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov’t Research Support, U.S. Gov’t, P.H.S. Review]. Journal of the American Society of Nephrology, 16(5), 1392–1403. doi:10.1681/ASN.2005010078.
Beinrohr, L., Dobo, J., Zavodszky, P., & Gal, P. (2008). C1, MBL-MASPs and C1-inhibitor: Novel approaches for targeting complement-mediated inflammation. [Research Support, Non-U.S. Gov’t Review]. Trends in Molecular Medicine, 14(12), 511–521. doi:10.1016/j.molmed.2008.09.009.
Carroll, M. C. (2004). The complement system in regulation of adaptive immunity. [Research Support, U.S. Gov’t, P.H.S. Review]. Nature Immunology, 5(10), 981–986. doi:10.1038/ni1113.
Fujita, T. (2002). Evolution of the lectin-complement pathway and its role in innate immunity. [Research Support, Non-U.S. Gov’t Review]. Nature Reviews. Immunology, 2(5), 346–353. doi:10.1038/nri800.
Hajishengallis, G., & Lambris, J. D. (2010). Crosstalk pathways between Toll-like receptors and the complement system. [Research Support, N.I.H., Extramural Research Support, U.S. Gov’t, P.H.S. Review]. Trends in Immunology, 31(4), 154–163. doi:10.1016/j.it.2010.01.002.
Hourcade, D., Liszewski, M. K., Krych-Goldberg, M., & Atkinson, J. P. (2000). Functional domains, structural variations and pathogen interactions of MCP, DAF and CR1. [Review]. Immunopharmacology, 49(1–2), 103–116.
Kaplan, A. P., Ghebrehiwet, B., Silverberg, M., & Sealey, J. E. (1981). The intrinsic coagulation-kinin pathway, complement cascades, plasma renin-angiotensin system, and their interrelationships. [Review]. Critical Reviews in Immunology, 3(1), 75–93.
Kavanagh, D., Richards, A., & Atkinson, J. (2008). Complement regulatory genes and hemolytic uremic syndromes. [Review]. Annual Review of Medicine, 59, 293–309. doi:10.1146/annurev.med.59.060106.185110.
Kemper, C., & Atkinson, J. P. (2007). T-cell regulation: With complements from innate immunity. [Research Support, Non-U.S. Gov’t Review]. Nature Reviews. Immunology, 7(1), 9–18. doi:10.1038/nri1994.
Kemper, C., Atkinson, J. P., & Hourcade, D. E. (2010). Properdin: Emerging roles of a pattern-recognition molecule. [Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov’t Review]. Annual Review of Immunology, 28, 131–155. doi:10.1146/annurev-immunol-030409-101250.
Kishore, U., & Sim, R. B. (2012). Factor H as a regulator of the classical pathway activation. [Research Support, Non-U.S. Gov’t]. Immunobiology, 217(2), 162–168. doi:10.1016/j.imbio.2011.07.024.
Kjaer, T. R., Thiel, S., & Andersen, G. R. (2013). Toward a structure-based comprehension of the lectin pathway of complement. [Research Support, Non-U.S. Gov’t Review]. Molecular Immunology, 56(3), 222–231. doi:10.1016/j.molimm.2013.05.220.
Klos, A., Tenner, A. J., Johswich, K. O., Ager, R. R., Reis, E. S., & Kohl, J. (2009). The role of the anaphylatoxins in health and disease. Molecular Immunology, 46, 2753–2766.
Klos, A., Wende, E., Wareham, K. J., & Monk, P. N. (2013). International Union of Pharmacology. LXXXVII. Complement peptide C5a, C4a, and C3a receptors. [Research Support, Non-U.S. Gov’t Review]. Pharmacological Reviews, 65(1), 500–543.
Makrides, S. C. (1998). Therapeutic inhibition of the complement system. [Review]. Pharmacological Reviews, 50(1), 59–87.
Marth, T., & Kelsall, B. L. (1997). Regulation of interleukin-12 by complement receptor 3 signaling. [Research Support, Non-U.S. Gov’t]. Journal of Experimental Medicine, 185(11), 1987–1995.
Mullins, R. F., Aptsiauri, N., & Hageman, G. S. (2001). Structure and composition of drusen associated with glomerulonephritis: Implications for the role of complement activation in drusen biogenesis. [Research Support, Non-U.S. Gov’t Research Support, U.S. Gov’t, P.H.S.]. Eye (London, England), 15(Pt 3), 390–395. doi:10.1038/eye.2001.142.
Ricklin, D., & Lambris, J. D. (2007). Complement-targeted therapeutics. Nature Biotechnology, 25(11), 1265–1275.
Ricklin, D., & Lambris, J. D. (2013). Progress and trends in complement therapeutics. Advances in Experimental Medicine and Biology, 735, 1–22. doi:10.1007/978-1-4614-4118-2_1.
Ricklin, D., Hajishengallis, G., Yang, K., & Lambris, J. D. (2010). Complement: A key system for immune surveillance and homeostasis. Nature Immunology, 11(9), 785–797.
Schwarz, M., Spath, L., Lux, C. A., Paprotka, K., Torzewski, M., Dersch, K., et al. (2008). Potential protective role of apoprotein J (clusterin) in atherogenesis: Binding to enzymatically modified low-density lipoprotein reduces fatty acid-mediated cytotoxicity. [Research Support, Non-U.S. Gov’t]. Thrombosis and Haemostasis, 100(1), 110–118. doi:10.1160/TH07-12-0737.
Singh, B., Su, Y. C., & Riesbeck, K. (2010). Vitronectin in bacterial pathogenesis: A host protein used in complement escape and cellular invasion. [Research Support, Non-U.S. Gov’t Review]. Molecular Microbiology, 78(3), 545–560. doi:10.1111/j.1365-2958.2010.07373.x.
Sjoberg, A. P., Trouw, L. A., & Blom, A. M. (2009). Complement activation and inhibition: A delicate balance. [Review]. Trends in Immunology, 30(2), 83–90. doi:10.1016/j.it.2008.11.003.
Tedesco, F., Pausa, M., Nardon, E., Introna, M., Mantovani, A., & Dobrina, A. (1997). The cytolytically inactive terminal complement complex activates endothelial cells to express adhesion molecules and tissue factor procoagulant activity. [Research Support, Non-U.S. Gov’t]. Journal of Experimental Medicine, 185(9), 1619–1627.
Trouw, L. A., & Daha, M. R. (2011). Role of complement in innate immunity and host defense. [Research Support, Non-U.S. Gov’t Review]. Immunology Letters, 138(1), 35–37. doi:10.1016/j.imlet.2011.02.014.
Wallis, R., Mitchell, D. A., Schmid, R., Schwaeble, W. J., & Keeble, A. H. (2010). Paths reunited: Initiation of the classical and lectin pathways of complement activation. [Research Support, Non-U.S. Gov’t Review]. Immunobiology, 215(1), 1–11. doi:10.1016/j.imbio.2009.08.006.
Walport, M. J. (2001a). Complement – First of two parts. New England Journal of Medicine, 344(14), 1058–1066. doi:10.1056/nejm200104053441406.
Walport, M. J. (2001b). Complement – Second of two parts. New England Journal of Medicine, 344(15), 1140–1144. doi:10.1056/nejm200104123441506.
Wyatt, R. J., McAdams, A. J., Forristal, J., Snyder, J., & West, C. D. (1979). Glomerular deposition of complement-control proteins in acute and chronic glomerulonephritis. [Research Support, U.S. Gov’t, P.H.S.]. Kidney International, 16(4), 505–512.
Zipfel, P. F. (2009). Complement and Immune defense: From innate immunity to human diseases. Immunology Letters, 126, 1–7.
Zipfel, P. F., & Skerka, C. (2009). Complement regulators and inhibitory proteins. Nature Reviews. Immunology, 9(10), 729–740.
Zipfel, P. F., Heinen, S., Jozsi, M., & Skerka, C. (2006). Complement and diseases: Defective alternative pathway control results in kidney and eye diseases. [Research Support, Non-U.S. Gov’t Review]. Molecular Immunology, 43(1–2), 97–106. doi:10.1016/j.molimm.2005.06.015.
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Kraiczy, P. (2016). Complement System. In: Parnham, M.J. (eds) Compendium of Inflammatory Diseases. Springer, Basel. https://doi.org/10.1007/978-3-7643-8550-7_219
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DOI: https://doi.org/10.1007/978-3-7643-8550-7_219
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