Fig. 2
Impairment of mitochondrial respiratory function in iNSCs derived from mutant MERRF-iPSCs. A A schematic diagram to depict the stages for cortical neuronal differentiation of MERRF-iPSCs. B Characterization of neural stem cells (NSCs) derived from MERRF-iPSCs after going through the neural-lineage differentiation by the immunofluorescence staining of neural stem cell marker, Nestin (Green). Nuclei were counterstained with Hoechst 33342 (blue). Scale bars, 100 μm. C Quantification of the Nestin fluorescent intensity in immunofluorescence image was normalized with nuclei counts. All the data are presented as a fold change of normal iNSCs (N iNSCs). D The protein expression patterns of NSCs marker genes in normal and MERRF-iNSCs lines. Quantification of the proteins in Western blots was normalized with β-actin. All data displayed as a fold of N iNSCs. E The protein expression levels of some subunits of respiratory enzyme complexes in normal and MERRF-iNSCs. Quantification of the proteins in Western blots was normalized with β-actin. All the data displayed as a fold change of N iNSCs. F Mitochondrial respiration rates of normal and MERRF-iNSCs were analyzed by a Seahorse XFe24 extracellular flux analyzer. G Quantitative data of the basal respiration rate, maximal, and ATP-coupled mitochondrial respiration rates of MERRF-iNSCs (M1Low, M1High, M2High, M3Low, and M3Med) as compared with those of N iNSCs. Data are presented as mean ± SEM, n = 3. *p < 0.05; **p < 0.01