FIGURE 6.

Electron transport chain defects in ReNcell VM 122 CAG neurons. A, The activities of complex I and II + III are significantly reduced in the 122 CAG line (*P < .05) but complex IV was unaffected compared across all lines at DD14. To further pinpoint the defect in mitochondrial respiration, the 30 CAG and 122 CAG lines were assayed for complex II and III and B, CoQ₁₀ status. CoQ₁₀ status is expressed as pmol/mg divided by CS activity. No significant difference was found in complex II activity or the electron carrier CoQ₁₀, but a significant reduction in complex III activity was detected in the 122 CAG neurons (*P < .05). All complex activities are normalized to CS activity to correct for mitochondrial enrichment of the samples, and normalized for total protein. Means were calculated for three samples generated from three independent differentiations per line. Data are shown as mean ± SEM. C, The relative levels of mitochondrial complex subunits were measured by Western blotting of labile subunits from complexes I‐V (green) vs GAPDH (red). D, Quantification of expression levels of individual complex subunits shown in C by band densitometry, normalized to GAPDH. No significant differences in expression were found. Data are shown as mean ± SEM