[The role of complement in physiology and pathology].
The complement system was discovered over one hundred years ago. It is an essential part of the innate immune system. A group of about 40 proteins assists in phagocytosis and stimulates inflammation. The complement system participates in the defense of an organism against different factors, e.g. microorganisms. There are three pathways of complement activation: the classical, lectin, and alternative. Activation of the complement system leads to the formation of a lytic macromolecule known as the membrane attack complex (MAC). The MAC may damage target cells in a process called bacteriolysis. The host organism is protected against the negative impact of autoimmunity by complement factor H (CFH). Recent experimental studies dealing with the regulation of the complement system suggest that this control process can be genetically determined. Mutations in genes encoding CFH (CFH polymorphism), factor B, and C2, can be crucial for a defective or insufficient regulation of the complement system. This paper surveys recent achievements on the structure and mechanisms of the complement system and shortly reviews the correlation between the complement function and pathogenesis of many diseases, including atypical hemolytic uremic syndrome (aHUS), membranoproliferative glomerulonephritis II (MPGN II), and age-related macular degeneration (AMD).
Klaska
Izabela
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Centrum Biologii Medycznej, Polska Akademia Nauk, Łódź, Poland.
Nowak
Jerzy Z
JZ
D000818:Animals; D003167:Complement Activation / Q000502:physiology; D051561:Complement C3 Convertase, Alternative Pathway / Q000502:physiology*; D017242:Complement Factor H / Q000737:chemistry / Q000502:physiology*; D003169:Complement Inactivator Proteins / Q000502:physiology; D015938:Complement Membrane Attack Complex / Q000737:chemistry / Q000502:physiology*; D003170:Complement Pathway, Alternative / Q000502:physiology; D003165:Complement System Proteins / Q000737:chemistry / Q000502:physiology*; D015432:Glomerulonephritis, Membranoproliferative / Q000276:immunology / Q000378:metabolism* / Q000473:pathology; D006463:Hemolytic-Uremic Syndrome / Q000276:immunology / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D008268:Macular Degeneration / Q000276:immunology / Q000378:metabolism* / Q000473:pathology
D003169:Complement Inactivator Proteins; D015938:Complement Membrane Attack Complex; D017242:Complement Factor H; D003165:Complement System Proteins; D051561:Complement C3 Convertase, Alternative Pathway