title: The complement cascade and renal disease.
abstract: Serum complement cascade, a part of innate immunity required for host protection against invading pathogens, is also a mediator of various forms of disease and injury. It is activated by classical, lectin, and alternative pathways that lead to activation of C3 component by C3 convertases, release of C3b opsonin, C5 conversion and eventually membrane attack complex formation. The tightly regulated activation process yields also C3a and C5a anaphylatoxins, which target a broad spectrum of immune and non-immune cells. The review discusses the involvement of the complement cascade in kidney disease pathogenesis and injury. The role of the complement pathways in autoantibody-mediated forms of glomerulonephritis (lupus nephritis, anti-glomerular basement membrane disease, anti-neutrophil cytoplasmic autoantibody-induced or membranoproliferative glomerulonephritis, membranous nephropathy), C3 glomerulopathy, atypical forms of hemolytic uremic syndrome, ischemic-reperfusion injury of transplanted kidney, and antibody-mediated renal allograft rejection are discussed. The disturbances in complement activation and regulation with underlying genetics are presented and related to observed pathology. Also promising strategies targeting the complement system in complement-related disorders are mentioned.
authors.affiliation: Department of Nephrology and Transplantation Medicine, Wroclaw Medical University, Wrocław, Poland, katarzynakoscielska@interia.pl.
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Kościelska-Kasprzak
authors.forename: Dorota
authors.initials: D
authors.lastname: Bartoszek
authors.forename: Marta
authors.initials: M
authors.lastname: Myszka
authors.forename: Marcelina
authors.initials: M
authors.lastname: Zabińska
authors.forename: Marian
authors.initials: M
authors.lastname: Klinger
mesh_terms: D000818:Animals; D000936:Antigen-Antibody Complex / Q000276:immunology*; D001323:Autoantibodies / Q000378:metabolism; D001485:Basement Membrane / Q000276:immunology; D003167:Complement Activation / Q000235:genetics; D003165:Complement System Proteins / Q000276:immunology*; D006084:Graft Rejection / Q000276:immunology*; D006801:Humans; D007113:Immunity, Innate / Q000235:genetics; D007518:Isoantibodies / Q000378:metabolism; D007674:Kidney Diseases / Q000235:genetics / Q000276:immunology*; D016030:Kidney Transplantation
chemical_list: D000936:Antigen-Antibody Complex; D001323:Autoantibodies; D007518:Isoantibodies; D003165:Complement System Proteins
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