Autoantibodies against alternative complement pathway proteins in renal pathologies.
Complement system activation plays an important role in several renal pathologies, including antibody-mediated glomerulonephritis, ischaemia-reperfusion injury of trasplanted kidneys or renal allograft rejection. Besides these conditions, alternative pathway abnormalities are directly involved in the pathogenesis of C3 glomerulopathies and atypical haemolytic uraemic syndrome. These abnormalities may be inherited or acquired, the latter as autoantibodies directed against the various components and regulators of the alternative complement pathway. The functional consequences of some of these antibodies and their association with these conditions are well known, whereas for other antibodies only isolated cases have been reported. This article describes the autoantibodies that target the alternative complement pathway proteins, their characteristics and their clinical relevance in renal pathologies.
Unidad de Inmunología, Hospital Universitario La Paz. IdiPAZ. CIBERER U-754, Madrid, España.
Pilar
P
Nozal
Unidad de Inmunología, Hospital Universitario La Paz. IdiPAZ. CIBERER U-754, Madrid, España. Electronic address: mltrascasa@salud.madrid.org.
Margarita
M
López-Trascasa
D001323:Autoantibodies / Q000097:blood*; D003176:Complement C3; D003170:Complement Pathway, Alternative; D003165:Complement System Proteins / Q000276:immunology*; D005921:Glomerulonephritis; D006801:Humans; D007674:Kidney Diseases / Q000276:immunology*; D016030:Kidney Transplantation; D015427:Reperfusion Injury
D001323:Autoantibodies; D003176:Complement C3; D003165:Complement System Proteins
Alternative pathway; Autoantibodies; Autoanticuerpos; C3; C3NeF; Factor B; Factor H; Factor I; Vía alternativa