Figure 7. Mutant horse and dog PrP induce locomotor dysfunction and shorter survival.

A-C, Locomotor performance. A, Flies expressing RaPrP-S174N pan-neurally show the same climbing performance (50% climbing at 26 days) than flies expressing RaPrP-WT (50% climbing at 27 days) and the reporter LacZ (50% climbing at 28 days). B, Flies expressing EqPrP-WT (50% climbing at 28 days) exhibit a similar locomotor profile than control flies expressing LacZ. However, flies expressing EqPrP-SQ167,168DE show a prominent decrease in climbing performance (50% climbing at 9 days). C, Flies expressing CaPrP-WT (50% climbing at 28 days) exhibit a similar locomotor profile than control flies expressing LacZ. In contrast, flies expressing CaPrP-D159N or -YD155,159NN show a prominent decrease in climbing performance (50% climbing at 7.5 days). The LacZ controls are the same in A-C because all climbing experiments were performed at the same time. D-F, Longevity. D, Flies expressing RaPrP-S174N pan-neurally show similar survival curves than flies expressing RaPrP-WT and the reporter LacZ. E, Flies expressing EqPrP-WT exhibit a similar survival profile than control flies expressing LacZ. However, flies expressing EqPrP-SQ167,168DE show a prominent reduction in viability. F, Flies expressing CaPrP-WT exhibit a similar survival profile than control flies expressing LacZ. In contrast, flies expressing CaPrP-D159N or -YD155,159NN show a prominent decrease in longevity. Notes: The LacZ controls are the same in A-C and D-F because all climbing and longevity experiments were performed at the same time. After Bonferroni post hoc correction, adjusted α = 0.00625. Not significant differences (p > 0.00625) are not indicated for clarity of the panels; *p < 0.00625; ** p < 0.001; *** p < 0.0001.