To test our CRISPR/Cas9-mediated gene editing approach, we excised the pathogenic GAA repeat mutations in the intron 1 of FXN in FRDA lymphoblasts utilizing our optimized protocol with Cas9 protein and UP4-DN4 guideRNAs in a ribonucleoprotein complex (Rocca et al., 2020). This evidence concerns the gene FXN and Friedreich ataxia.