Article
Membranoproliferative glomerulonephritis with C3NeF and genetic complement dysregulation.
Pediatric nephrology (Berlin, Germany) - 1 Mar 2011
Leroy Valérie, Fremeaux-Bacchi Véronique, Peuchmaur Michel, Baudouin Véronique, Deschênes Georges, Macher Marie-Alice, Loirat Chantal
Abstract excerpt
The development of membranoproliferative glomerulonephritis (MPGN) is associated with uncontrolled activation of the complement alternative pathway. This dysregulation is related either to C3 nephritic factor (C3NeF), an auto-antibody directed against the alternative C3 convertase, or to homozygous loss-of-function mutation of the complement regulatory protein factor H. Heterozygous mutations in the genes coding...
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