Figure 3

GAN mice present persistent motor deficits. Motor functions were evaluated with a Grip Strength test (A), and a Rotarod test (B). Sensory deficits were recorded with a Hot plate test (C) and Von Frey filaments test (D). Each test was performed every 4 weeks over a 72 week-period (n = 15 mice per genotype). The scores of individual mice are represented at 72 weeks with the mean score represented by a bar for each genotype. The average score was also represented over time for the Forelimb grip test, to show the statistical significance of motor impairment in the GAN mice from 60 weeks of age (two-way ANOVA with Bonferroni post-test: *, p < 0,5; **, p < 0,01 and ***, p < 0,001). Gait analysis of the GAN mice at 72 weeks of age (E). The stride length (i), the width between the right and left paws (ii) and the overlap between the forepaws and hindpaws was measured for each mouse (n = 15 per genotype) and averaged (bar). Analysis of the other GAN^ex3-5 model (GAN ^YY) did not reveal any motor deficits within the first year of age (F) (n = 10 per genotype).