Fig. 1: Frataxin is in close proximity to mitochondrial respiratory chain complexes I, II and III in healthy hiPSC-derived CMs and hiPSC-derived NPCs.

On the top of both panels, representative confocal images of in situ proximity ligation assay (PLA) performed in hiPSC-CMs (left panel) and hiPSc-NPCs (right panel) using an anti-frataxin antibody in combination with antibodies against the respiratory chain complexes I, II or III. Scale bar: 30 μm. Nuclei were counterstained with DAPI (blue). Red fluorescent dots were quantified and reported as PLA dots/cell (on the bottom). Analyzed data, reported as the means ± SEM, derived from three independent experiments in which at least five different fields were acquired. Statistical significance was determined using unpaired t-test (*p ≤ 0.05, **p ≤ 0.01, ***p ≤ 0.001 compared to healthy control cell lines).