Figure 3. Bezafibrate administration to ΔCOX10 mice induces PGC-1α expression in skeletal muscle resulting in delayed onset of the myopathy.

A: Survival curve of female ΔCOX10 on the bezafibrate diet mice in comparison to ΔCOX10 mice on a regular diet (n=7 for each group). No mice of in wild-type groups (bezafibrate diet or regular diet) died in the observed timeframe. B: Treadmill performance test at different ages for ΔCOX10 and wild-type mice on a bezafibrate diet. ΔCOX10 and wild-type mice on a regular diet were used as reference (n= 3 for each group). At the 3 months time point, statistical significance was reached between ΔCOX10 on the standard diet and each of the other groups. At the 6 months time point, significance was reached between ΔCOX10 on the bezafibrate diet and each of the other groups. C: Cytochrome c oxidase (COX) activity and citrate synthase activity in muscle homogenates of 3 months old female ΔCOX10 and wild-type mice on a bezafibrate and regular diet (n=3 for each group). D: Histology of the biceps femoris muscle from 3 months old female mice showing ΔCOX10 and wild-type mice on a bezafibrate and regular diet. SDH staining is shown to indicate the mitochondrial proliferation. COX and combined COX/SDH staining highlight the degree of COX deficiency. E: Quantification of ATP in the biceps femoris muscle from 3 months old ΔCOX10 and wild-type mice on a bezafibrate and regular diet (n=2 for each group). F: Relative expression of PGC-1α, PGC-1β and PPARα/δ and γ in skeletal muscle of 3 months old female ΔCOX10 and wild-type mice on a bezafibrate diet. ΔCOX10 and wild-type mice on a regular diet were used as reference.