Complement and complement deficiencies.
The complement system provides a first line of defense and mediates a large variety of cellular and humoral interactions within the immune response, including chemotaxis, phagocytosis, cell adhesion, and B-cell differentiation. The system involves more than 30 serum components and numerous cell surface regulators and receptors. Similar to the blood clotting system, complement activation is initiated through a series of complex activation cascades involving enzymatic cleavage. Three independent complement activation cascades, the classical, the alternative, and the lectin pathway, have been described. The liver is the main site of biosynthesis for most of the serum components of complement and diseases of the liver can lead to alterations of the normally stable plasma levels of complement. Deficiencies of single components can lead to a broad variety of secondary diseases, caused by either imbalanced activation or defects in the humoral or cellular response to microbial infections.
Whaley
K
K
Department of Microbiology and Immunology, University of Leicester, England.
Schwaeble
W
W
D015926:Complement C3a / Q000172:deficiency / Q000276:immunology*; D003179:Complement C3b / Q000172:deficiency / Q000276:immunology*; D015936:Complement C5a / Q000172:deficiency / Q000276:immunology*; D003171:Complement Pathway, Classical / Q000502:physiology*; D006801:Humans; D007107:Immune System / Q000502:physiology; D007111:Immunity, Cellular / Q000502:physiology*; D011951:Receptors, Complement / Q000276:immunology / Q000502:physiology
D011951:Receptors, Complement; D015926:Complement C3a; D003179:Complement C3b; D015936:Complement C5a