Complement regulation.
Because of its strong potential for generating inflammation and causing tissue destruction the complement system has to be kept strictly under control. Cells of the host need special protection against the cytolytic complement system. This paper will describe how inappropriate activation of complement in the fluid phase is prevented and how viable human blood cells defend themselves against being destroyed and cleared away by the complement system. Since disturbances in complement regulation occasionally result in disease a brief reference will be made to two of the syndromes caused by complement regulator deficiency, hereditary angioedema (HAE) and paroxysmal nocturnal hemoglobinuria (PNH).
Department of Bacteriology and Immunology, Haartman Institute, Helsinki University Central Hospital, Finland. meri@helsinki.fi
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D000799:Angioedema / Q000097:blood / Q000235:genetics; D000818:Animals; D015703:Antigens, CD / Q000502:physiology; D018958:CD55 Antigens / Q000502:physiology; D002352:Carrier Proteins / Q000502:physiology; D051152:Clusterin; D037222:Collectins; D003167:Complement Activation / Q000502:physiology*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency / Q000235:genetics; D017242:Complement Factor H / Q000172:deficiency / Q000235:genetics; D003169:Complement Inactivator Proteins / Q000502:physiology; D003165:Complement System Proteins / Q000502:physiology; D004789:Enzyme Activation; D005246:Feedback; D005799:Genes, Dominant; D015432:Glomerulonephritis, Membranoproliferative / Q000235:genetics / Q000662:veterinary; D006023:Glycoproteins / Q000502:physiology; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood / Q000235:genetics; D006801:Humans; D007473:Ion Channels / Q000502:physiology; D051916:Membrane Cofactor Protein; D008562:Membrane Glycoproteins / Q000502:physiology; D008954:Models, Biological; D018832:Molecular Chaperones; D009895:Opsonin Proteins / Q000502:physiology; D011951:Receptors, Complement / Q000502:physiology; D013552:Swine; D013553:Swine Diseases / Q000235:genetics; D019096:Vitronectin
D015703:Antigens, CD; D018958:CD55 Antigens; D002352:Carrier Proteins; D051152:Clusterin; D037222:Collectins; D003174:Complement C1 Inactivator Proteins; D017242:Complement Factor H; D003169:Complement Inactivator Proteins; D003165:Complement System Proteins; D006023:Glycoproteins; D007473:Ion Channels; D051916:Membrane Cofactor Protein; D008562:Membrane Glycoproteins; D018832:Molecular Chaperones; D009895:Opsonin Proteins; D011951:Receptors, Complement; D019096:Vitronectin; C497857:CD46 protein, human; C493495:CFH protein, human; C495242:CLU protein, human; C086481:cationic protein, urate-calcium oxalate stone, human