Lu et al. (2012) reported that the presence of mutated TDP-43 protein in transfected NSC-34 cells causes morphological abnormalities, reduces complex I activity and determines a loss of transmembrane potential. In particular NSC-34 cells, following transfection with the mutated protein of interest, have been used to study the pathogenic mechanisms of ALS, including mitochondrial dysfunction (Muyderman et al., 2009). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.