Complement activity is regulated in C3 glomerulopathy by IgG-factor H fusion proteins with and without properdin targeting domains.

ABSTRACT: C3 glomerulopathy is characterized by accumulation of complement C3 within glomeruli. Causes include, but are not limited to, abnormalities in factor H, the major negative regulator of the complement alternative pathway. Factor H-deficient (Cfh^-/-) mice develop C3 glomerulopathy together with a reduction in plasma C3 levels. Using this model, we assessed the efficacy of two fusion proteins containing the factor H alternative pathway regulatory domains (FH_1-5) linked to either a non-targeting mouse immunoglobulin (IgG-FH_1-5) or to an anti-mouse properdin antibody (Anti-P-FH_1-5). Both proteins increased plasma C3 and reduced glomerular C3 deposition to an equivalent extent, suggesting that properdin-targeting was not required for FH_1-5 t

SUBMITTER: Gilmore AC 

PROVIDER: S-EPMC7863913 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature