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Complement Factor H-Related 5-Hybrid Proteins Anchor Properdin and Activate Complement at Self-Surfaces

Chen, Qian ; Manzke, Melanie ; Hartmann, Andrea ; Büttner, Maike ; Amann, Kerstin ; Pauly, Diana ; Wiesener, Michael ; Skerka, Christine ; Zipfel, Peter F.



Abstract

C3 glomerulopathy (C3G) is a severe kidney disease for which no specifictherapy exists. The causes of C3G are heterogeneous, and defective complement regulation is often linked to C3G pathogenesis. Copy number variations in the complement factor H-related (CFHR) gene cluster on chromosome 1q32 and CFHR5 mutant proteins associate with this disease. Here, we identified CFHR5 as a pattern ...

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