Fig. 5.

FXN expression rescue of FRDA hiPSC-hvCTS model. a FXN (i) transcript and (ii) protein expression (normalized to GAPDH expression) and (iii) representative Western blot image of FRDA-hiPSC-hvCTS after Lv-FXN transduction relative to Lv-RFP control (n = 3–4). Transcript and protein data were generated from 3 to 4 independent differentiation batches, respectively. b Representative contractile force traces on days 17–18 for FRDA-hiPSC-hvCTS transduced with Lv-FXN with Lv-RFP as control. c (i) Developed force, (ii) contractile rate, and (iii) relaxation rate generated at 1 Hz pacing in FRDA-hiPSC-hvCTS transduced with Lv-FXN (n = 10) relative to Lv-RFP control (n = 8). Data were generated by 4 independent batches of cell differentiation. FXN expression data are shown as mean ± SEM, and force generation data are shown as median with interquartile range. Statistical significance indicated by *p < 0.05 and **p < 0.01