The significant inhibitory effects on PrPSc propagation of the Cyp46A1 activator EFV in in vitro models of prion infection, and the reduced expression of Cyp46A1 in prion-infected mice, sCJD patients and prion-infected neuronal cells, inspired us to evaluate EFV effects in an animal model of prion disease. Here, CYP46A1 is linked to prion disease.