Activation of SIRT1 prevents energetic failure, promotes mitochondrial respiration and increases antioxidant defense in Abcd1− mice. (a–n) Energetic failure, antioxidant defense and mitochondrial respiration were measured in the spinal cord of 12-month-old (a, c, e, f, g and k) WT (n=5), Abcd1−(n=6) and Abcd1− mice treated with RSV (Abcd1−+RSV) (n=6) and in 12-month-old (b, d, h, I, j and l) WT (n=5), Abcd1− (n=6) and Abcd1−mice with transgenic overexpression of SIRT1 (Abcd1−/SIRT1Tg) (n=6). (a, b) ATP levels. Data are represented as fold change respect to WT mice. (c, d) NAD+, NADH and NAD+/NADH ratio. Data are represented as fold change with respect to WT mice. (e, h) PK activity. PK activity is represented as fold change with respect to WT mice. (f, i) GSH content. (g, j) GR activity. (k, l) Ex vivo mitochondrial respiration analysis performed on permeabilized sections of spinal cord. (C.I= complex I. C.II= complex II). Values are expressed as mean±S.D. (*P<0.05, **P<0.01 and ***P<0.001, one-way ANOVA followed by Tukey's HSD post hoc)