Research Paper Volume 9, Issue 4 pp 1341—1350

Mitochondrial replacement in an iPSC model of Leber’s hereditary optic neuropathy

Figure 2. LHON disease phenotype in iPSC-derived RGCs can be reversed in corrected cybrid lines. (A) Efficiency in RGC differentiation as assessed by the % of THY1.1 positive cells obtained post MACS sorting for control, LHON and corrected cybrid lines. Data are expressed as mean + SEM of independent samples (n=11 for control, n=8 for LHON and n=8 for corrected cybrid, expressed as pooled data of experimental repeats and biological repeats (3 clones)). (B) TUNEL assay revealed increased susceptibility to apoptosis in LHON RGCs and reversal in corrected cybrid lines. Data are expressed as mean of each clone, n = 3 clones, error bars = mean ± SEM. (C) Quantification of mitochondrial superoxide levels using MitoSOX in control, LHON and corrected cybrid RGCs. Error bars = ± SEM, n = 3 clones. Statistical significance was established by one way ANOVA followed by Dunnett’s test for multiple comparisons, ** p<0.01, * p<0.05, ns: not significant.