Fig 2: TAT-MTScs-FXN is processed and functional in mice muscle (A) A representative image of frataxin present in mitochondrial fractions obtained from mice treated for 3 weeks—twice a week—with 10 mg/kg TAT-MTScs-FXN (lanes 1–6) or vehicle (lanes 7–8 where frataxin is almost undetectable). The same protocol was executed by administrating 4 mg/kg (not shown). Each lane (1–6) represents individual mouse. Unprocessed external TAT-MTScs-FXN protein was run in parallel (lane 9). Lower panel shows the levels of COX-IV that was used as internal mitochondrial marker. The frataxin levels normalized against COX-IV are shown in (B). Dashed line indicates the frataxin levels of heterozygous, healthy mice. Bars represent mean ± S.E.M., n = 4. In (C), the activity of mitochondrial complex II (succinate dehydrogenase) is shown. Values were obtained in samples of muscle mitochondrial fractions from mice treated with 4 and 10 mg/kg of TAT-MTScs-FXN and vehicle. Dashed line indicates the frataxin values for heterozygous, healthy mice. Results are presented as ?OD at 0–10 min. per total protein (bars represent mean ± S.E.M., n = 4, P < 0.05,anova).

Fig 3: LSDP5 reduces mitochondrial damage in LO2 lipotoxicity Model. (A) MMP rates were detected by JC-1 kit. (B) mRNA expression levels of Cytc, Cox IV and CPT1a were measured by reverse transcription-quantitative polymerase chain reaction. (C and D) Protein expression levels of Cytc, Cox IV and CPT1a were measured by western blot assay; ß-actin was used as a loading control and for normalization. *P<0.05 and **P<0.01 vs. Control; ^P<0.05 vs. Model. Cox IV, cytochrome c oxidase subunit IV; CPT1a, carnitine palmitoyltransferase 1a; Cytc, cytochrome c; LSDP5, lipid storage droplet protein 5; M, model; MMP, mitochondrial membrane potential; NC, negative control.