In fact, in skeletal myocytes differentiated from induced pluripotent stem cells (iPSCs) of C9ORF72-ALS patients, the occurrence of DPR proteins is accompanied by abnormalities in the expression of mitochondrial genes and a high susceptibility to oxidative stress, as well as increased expression and aggregation of TAR DNA-binding protein 43 (TDP-43) [47]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.