MtDNA and protein levels are increased in PLP‐tg66/66 spinal cords at 6 weeks of age. mtDNA content in 2‐ and 6‐weeks old mice is expressed as the ratio of mtDNA (cytb) to nuclear DNA (CEBP) (A). CxI subunits NDUFA9 and NDUFB8, CxII, CxIII, CxIV, CxV, VDAC, ACO2 and DLDH protein expression in 6‐week‐old mice (B). Relative gene expression of Pgc‐1α, Tfam, Nrf1, Nrf2 and Rip‐140 in 6‐week‐old mice (C). Relative protein expression of DRP1, Fis1, OPA1 and MFN2 in 6‐week‐old mice spinal cord (D). Representative blots are shown. The protein level is expressed as a fold increase of the control and in reference to γ‐tubulin as a loading marker. Two‐week‐old mice n = 7/genotype; 6‐week‐old mice, WT n = 6, PLP‐tg66/66 n = 7. CTL fibroblasts n = 4; PLP1‐duplicated PMD fibroblasts n = 4. Values are expressed as the mean ± SD. Statistical analysis was performed using Student's t‐test; *P < 0.05, **P < 0.01, ***P < 0.001.