Fig. 1.

NR ​+ ​PT ​+ ​IBU delay loss of functional performance and extend survival in SOD1^G93A and FUS^R521C mice. (a) Neurological score. (b) Rotarod test. (c) Survival. Treatment arms were as follows: physiological saline-treated transgenic mice (controls), SOD1 ^G93A or FUS^R521C mice treated with NR and PT, SOD1 ^G93A or FUS^R521C mice treated with IBU, and SOD1 ^G93A or FUS^R521C mice treated with NR, PT and IBU. The number of mice tested per week (tests a and b) was identical to that displayed in the survival experiments (c) for each experimental group. Both neuromotor tests (a, b) were performed twice a week. On the days of the week in which the animals were not scored, they were trained in the rotarod. Control experiments performed in WT mice (n ​= ​10) rendered a neurological score of 0 and a performance time of 1200 ​s in the rotarod test in all cases. Taking into account the % of animals that died during disease progression, to assess the neurological score and the rotarod test, their number was increased so that n (15 mice) was approx. the same both at onset and at advanced progression. A one-way analysis of variance (ANOVA) was used to make comparisons among the different treatments at each time point. Different letters indicate statistical differences, P ​< ​0.05 (a, b). Survival data were analyzed with Kaplan-Meier curves and LogRank (Mantel-Cox) test (∗P ​< ​0.01 comparing all groups versus controls; ⁺P ​< ​0.01 comparing mice treated with NR ​+ ​PT ​+ ​IBU versus mice treated with NR ​+ ​PT) (c).