Remarkably, the male hFH-FHR5mut phenotype recapitulated the key features of CHFR5 nephropathy: normal FH function, intact plasma C3 regulation, abnormal glomerular C3 deposition in the absence of IgG, and the development of electron-dense deposits within the GBM and mesangial matrix. The gene discussed is C3; the disease is Nephropathy.