Homozygote mice (for consistency we will adopt the Song nomenclature approach, thus C3N/N) display all the hallmarks of aHUS, that is, acute kidney injury (elevated BUN, proteinuria, and hematuria), thrombocytopenia, anemia and thrombotic microangiopathy (evidenced in renal histology) associated with significant AP dysregulation (systemic changes in C3, C5 turnover as well as significant glomerular C3 deposits in the glomerulus). Here, C5 is linked to thrombotic microangiopathy.