Tg(PrP-P101L) mice, which express high levels of mouse PrP-P101L, the analogous mutation to human PrP-P102L, have been shown to spontaneously develop prion disease-like diseases, with amyloid plaques, spongiform degeneration, and gliosis in their brains (Table 3) [77]. Here, PRNP is linked to prion disease.