A mouse model of non-Shiga toxin-associated haemolytic uraemic syndrome

Impaired control of the complement system activation due to mutations in complement factor H (CFH) has been described in two apparently unrelated human diseases, membranoproliferative glomerulonephitis type II (MPGN2) and non-Shiga toxin-associated haemolytic uraemic syndrome (non-Stx-HUS). Mouse models of these diseases have been developed by Pickering et al., by knocking-out Cfh gene (MPGN2) and by subsequently transferring a mutated Cfh gene in the Cfh−/− background (non-Stx-HUS). The data obtained from the two models provided precious information to clarify the mechanisms that cause the disparate phenotypes underlying CFH genetic defect.