Humanized C3 Mouse: A Novel Accelerated Model of C3 Glomerulopathy

We developed a novel, rapidly-progressing, severe murine model of C3G by replacing the mouse C3 gene with the human C3 homologue using Velocigene® technology. We conducted functional, histological, molecular and pharmacologic assays to characterize the presentation of renal disease and useful pharmacologic interventions in the humanized C3 (C3hu/hu) mice. 3 separate experiments are included. The first profiles livers from WT and humanized C3 (huC3) mice, N = 3 each. Experiment two replicated the experiment in kidneys (N = 3 for WT, N = 6 for huC3). Experiment three profiles kidneys from WT mice (N =5), huC3 mice treated with control antibody (N=3), and huC3 mice treated with an anti-C5 mAb (N = 4).