Figure 6.

Early onset of behavioral alterations and progressive motor decline in TBA2.1 mice. Behavioral testing in automated home-cage environment reveals decline of free feeding (A, black line) and drinking (A, gray line) over time, as well as significantly altered rearing behavior across 6 d of individual recordings (A, small panels) in HOM TBA2.1 (gray actigrams) compared with age-matched WT littermates (black actigrams); data are expressed as percentage of WT littermate scores (feeding, drinking) or show number of rearing events (small panels) and were analyzed by two-way repeated-measures ANOVA and represent means; ***p < 0.001; n ≥ 7 animals per group. Abnormal performance of HOM TBA2.1 in primary neurobehavioral assessment (B, black line; percentage of animals affected per group) and in the rotarod test (B, gray line; percentage of WT littermate scores) increases over time; n ≥ 7 animals per group. A clear loss of the PPI of the auditory startle reflex in HOM TBA2.1 (C, black bars) compared with age-matched WT littermates (C, open bars) across 72–84 dB prepulse intensity (aged 28 d) is detected; *p < 0.05, **p < 0.01; data were analyzed by two-way repeated-measures ANOVA followed by Bonferroni's post hoc test and represent means ± SEM; n ≥ 14 animals per genotype.