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. 2017 Dec 4;27(23):3626–3642.e6. doi: 10.1016/j.cub.2017.10.054

Figure 1.

Figure 1

Behavioral Assessment of DRPLA Mice

(A–D) Grip strength analysis revealed the progression of degenerative decline in ATN1-FL-65Q mice (red) compared to wild-type mice (WT, black) and ATN1-FL-26Q (blue) over time as measured by repeated-measures two-way ANOVA. This was evidenced by significant interaction between age (v1) and genotype (v2) (Xp < 0.05,XXp < 0.01, XXXp < 0.001) when measuring both limbs (A and B). Hereby the progression was stronger in males signified by stronger interaction in both limbs (B) compared to females (A). In addition, males showed progression when only forelimb grip strength was measured (D). In contrast, females showed overall decreased non-progressive grip strength levels for fore limbs (C). Individual values are given as mean ± SEM and significance levels for individual time points are assigned above with ∗p < 0.05, ∗∗p < 0.01, and ∗∗∗p < 0.001.

(E) Thigmotaxis as a measure of anxiety was evaluated for the first 5 min after introduction to the open field by assessing the time 10-week-old males and females spent in the outer zone. The ATN1-FL-65Q (65Q, red) line showed a significantly higher tendency to remain close to the walls of the arena as compared to the wild-type (wt; black) and ATN1-FL-26Q (26Q; blue) mice. Automatic quantification using EthoVision 7XT software. One-way ANOVA, ∗∗p < 0.01.

(F) General activity was assessed in females at 10 and 14 weeks evaluating the distance traveled from 5 to 25 min after introduction to the open field. ATN1-FL-26Q (blue) mice were significantly more active compared to the wild-type (WT; black) at 10 weeks. This difference was more pronounced at 14 weeks of age with both the ATN1-FL-26Q and ATN1-FL-65Q (red) lines being more active than the wild-type, while ATN1-FL-65Q mice were less active than ATN1-FL-26Q. An interaction with age was not observed (repeated-measures two-way ANOVA, v1, genotype; v2, age). Automatic quantification using EthoVision 7XT software. One-way ANOVA ∗p < 0.05, ∗∗p < 0.01, and ∗∗∗p < 0.001.

(G) In gait analysis at 18 weeks of age, ATN1-FL-26Q shows a typical coordinated and regular footprint placing the hind paws (red) close to front paws (blue). In contrast, ATN1-FL-65Q shows an uncoordinated pattern typical for ataxia.

See also Figure S1.