Figure 6

Treatment with TAT-ORF improves functionality of patients’ mitochondria mutated in the C6ORF66 (NDUFAF4) assembly factor. The effect of TAT-ORF treatment was tested on the following: amount of the cells (A); mitochondrial content (B); production of total ATP in the cells, normalized to the amount of cell ATP/methylene blue (MB) (C) and normalized to mitochondrial content ATP/MitoTracker Green (MTG) (D); mitochondrial membrane potential TMRE/MTG (E); and production of free radicals DCF/MTG (F). The experiment was performed with cells from patient F511 treated with TAT-ORF (0.022 µg/µL, final concentration) for 24 h. All measures were performed as described in Materials and Methods. Data are presented as the average of four to five repeats ± SD. *p < 0.5.