Fig. 2

Disruption of polyamine catabolism provokes the development of age-dependent, progressive ataxia. Development of ataxia was monitored by comparing the CAS, rotarod latency to fall and gait of Smox/Sat1-dKO (n = 13) to those of Smox-KO (n = 9), Sat1-KO (n = 8), and Wt (n = 11) mice. a The CAS of Smox/Sat1-dKO were not significantly different than Wt, Smox-KO, and Sat1-KO mice at 8 weeks of age. The CAS of Smox/Sat1-dKO mice were significantly higher than those of Wt, Smox-KO, and Sat1-KO mice as early as 10 weeks of age (p = 0.00245, p = 0.00497, and p = 0.0000239). The CAS scores continued to increase, remaining significantly higher in Smox/Sat1-dKO compared to Wt, Smox-KO, and Sat1-KO in 12 weeks (p = 0.00586, p = 0.00817, and p = 0.00586) and 14 weeks (p = 0.00274, p = 0.00217, and p = 0.00372) of age. b Rotorod studies revealed that only the Smox/Sat1-dKO mice exhibited a progressive and significant reduction in their latency to fall when results at 12 weeks (p = 0.00423) and 14 weeks (p = 3.37 × 10⁻⁷) of age were compared to those of 8-week-old mice. The Smox/Sat1-dKO mice had a significantly shorter latency to fall time compared to Wt, Smox-KO, and Sat1-KO mice as early as 8 weeks of age (p = 0.000846, p = 0.00928, and p = 0.0297). The results are shown as the means ± SD. For CAS and rotorod results, (*) denotes p < 0.05 and (**) denotes p < 0.01 for comparison to Wt. For rotorod results, (+) and (#) denote p < 0.01 and p < 0.001 for comparison to 8-week-old Smox/Sat1-dKO mice. c Gait analyses of Wt versus Smox/Sat1-dKO mice indicate that there were significant differences in both stance (p = 0.0346) and stride (p = 0.0334) values on week 8 and significant differences in stride values on week 12 (p = 0.000404). For gait analysis results, (*) denotes p < 0.05 and (**) denotes p < 0.01