Extended Data Fig. 8: Female and male Etfdh-/- mice display myopathy.
a) Representative western blot analysis of ETFDH protein levels in Skm, brain and heart from wt and Etfdh -/- mice. ETFDH is absent specifically in muscle. b) Weight loss after fasting (150 days old mice) of wild-type (wt) in male and female Etfdh-/- mice. n = 10 mice/genotype. c–g) Motor behavior assays in female (♀) and male (♂) mice. RotaRod max speed (c), time for 1 min at fixed rpm (d) time for 1 min at 32 rpm (e) and number of attempts to achieve 1 min at fixed speed (f) in 6 months-old wt and Etfdh -/- mice. In g, 2 limb hanging test in 6 months-old wt and Etfdh -/- mice. n = 11 animals/genotype (6 females and 5 males). h) Immunofluorescence from wt and ETFDH-ko myoblasts. Red, mitochondria: β-F1-ATPase; blue, nuclei: DAPI; green, lipid droplets: bodipy. Images are representative of 10 pictures/condition. Results are shown as the mean ± SEM of the indicated n. *, **, ***, **** p < 0.05; 0.01, 0.001 and 0.0001 when compared to wt by two-tailed Student’s t-test (b, g), and two-way ANOVA with Sidak’s test (c-f).
