<i>PANK2</i> mutations result in increased rate of ROS production, depletion of cellular GSH and higher oxidative stress.

<p>A) Representative images of glutathione levels in control and patient-derived neurons measured by monochlorobimane (MCB) fluorescence in live cells. B) Quantification of MCB fluorescence in iPSCs and neuronal cells showed reduced glutathione levels in patient-derived cells and lower levels in iPSCs, (Bⁱⁱ shows pooled data). C) Basal rates of ROS production were quantified in neurons using the dihydroethidium assay. D) Quantification showed an increased rate of ROS production in patient cells compared to controls and in all cell lines in response to 30 minutes pre-incubation with iron chelator DFO (Dⁱⁱ depicts pooled data from Dⁱ). E) The level of lipid peroxidation was quantified in the neuronal cells using the ratiometric dye BODIPY C11 and two of the patient-derived neuronal cells displayed significantly higher levels of lipid peroxidation (pooled data shown in Eⁱⁱ). Scale bar represents 20 μm. Significance was calculated via one way ANOVA with post-hoc Tukey’s HSD correction for multiple comparisons *p<0.05, **p<0.01, ***p<0.001, ns not significant.