Fig. 5.

Transgenic mouse models of inherited prion disease. The open reading frame of the PRNP gene is represented in the centre, and the mutations that have been modelled in transgenic animals are shown above and below. On the left, the species PrP sequence of the transgene is shown, i.e., hamster, bank vole, mouse, and bovine sequences as well as chimeric constructs, such as human/mouse. The amber symbols next to the mutant indicate that the model developed a spontaneous disease and the green symbol indicates that there was a distinct neuropathological phenotype either spontaneously or following inoculation with prions. In one study, the clinical and neuropathological aspects were not assessed (open symbol). The mouse lines with no symbol did not develop spontaneous disease and did not show distinct neuropathology Blue or red letters are simply for orientation, to align with the point mutation shown in the centre