Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome.
The alternative pathway of complement is implicated in the pathogenesis of several renal diseases, such as atypical hemolytic uremic syndrome, dense deposit disease and other forms of C3 glomerulopathy. The underlying complement defects include genetic and/or acquired factors, the latter in the form of autoantibodies. Because the autoimmune forms require a specific treatment, in part different from that of the genetic forms, it is important to detect the autoantibodies as soon as possible and understand their characteristics. In this overview, we summarize the types of anti-complement autoantibodies detected in such diseases, i.e. autoantibodies to factor H, factor I, C3b, factor B and those against the C3 convertases (C3 nephritic factor and C4 nephritic factor). We draw attention to newly described autoantibodies and their characteristics, and highlight similarities and differences in the autoimmune forms of these diseases.
MTA-ELTE Lendület Complement Research Group, Department of Immunology, Eötvös Loránd University, Budapest, Hungary. Electronic address: mihaly.jozsi@gmx.net.
Mihály
M
Józsi
Junior Research Group for Cellular Immunobiology, Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany.
Stefanie
S
Reuter
Unidad de Inmunología, Hospital Universitario La Paz, IdiPAZ, Madrid, Spain.
Pilar
P
Nozal
Unidad de Inmunología, Hospital Universitario La Paz, IdiPAZ, Madrid, Spain; CIBERER U754, Madrid, Spain.
Margarita
M
López-Trascasa
CIBERER U754, Madrid, Spain; Unidad de Investigación, Hospital Universitario La Paz, IdiPAZ, Madrid, Spain.
Pilar
P
Sánchez-Corral
Research Laboratory, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
Zoltán
Z
Prohászka
MTA-ELTE Immunology Research Group, Department of Immunology, Eötvös Loránd University, Budapest, Hungary.
Barbara
B
Uzonyi
D065766:Atypical Hemolytic Uremic Syndrome / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D001323:Autoantibodies / Q000096:biosynthesis* / Q000235:genetics / Q000276:immunology; D050577:Complement C3-C5 Convertases / Q000037:antagonists & inhibitors / Q000235:genetics / Q000276:immunology; D003179:Complement C3b / Q000037:antagonists & inhibitors / Q000235:genetics / Q000276:immunology; D011415:Complement Factor B / Q000037:antagonists & inhibitors / Q000235:genetics / Q000276:immunology; D017242:Complement Factor H / Q000037:antagonists & inhibitors / Q000235:genetics / Q000276:immunology; D003165:Complement System Proteins / Q000235:genetics / Q000276:immunology; D005340:Fibrinogen / Q000037:antagonists & inhibitors / Q000235:genetics / Q000276:immunology; D015870:Gene Expression; D005922:Glomerulonephritis, IGA / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D006801:Humans
D001323:Autoantibodies; C058872:complement 4 nephritic factor; D003179:Complement C3b; D017242:Complement Factor H; D005340:Fibrinogen; D003165:Complement System Proteins; D050577:Complement C3-C5 Convertases; D011415:Complement Factor B
Atypical hemolytic uremic syndrome; Autoantibodies; C3 convertase; C3 glomerulopathy; C3 nephritic factor; Complement regulation; Factor B; Factor H; Kidney disease