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. 2020 Nov 4;21(21):8251. doi: 10.3390/ijms21218251

Figure 8.

Figure 8

Seahorse assay in fibroblasts isolated from three FRDA patients and two healthy control subjects. (A) Representation of the oxygen consumption rate (OCR) in fibroblasts either non-transduced, or transduced with FXN I, FXN II or GFP as a positive transduction control. Patient cells transduced with frataxin isoforms seem to perform better on the assay, getting closer to the healthy cell OCR values. The X-axis plots different measurements over time (rates). (B) Relative quantification of the values of ATP-coupled respiration and of maximal respiratory capacity. Significant improvements in maximal respiratory capacity can be detected in patient fibroblasts after transduction with both frataxin isoforms. (C) Relative quantification of the values of basal respiratory capacity and spare capacity in both control and patient fibroblasts transduced with both isoforms. Data are represented as mean ± SEM and were analyzed with a one-way ANOVA with post hoc Dunnett multiple comparisons test from at least three independent experiments per cell line (n = 3). * p < 0.05; *** p < 0.001; **** p < 0.0001.