Aberrant cytoplasmic accumulation and aggregation of TDP-43 in MNs is a prevalent pathology in ALS patients, both in sporadic and familial cases with TDP-43 mutations (Sagner and Briscoe, 2019; Arai et al., 2006; Sreedharan et al., 2008; Lagier-Tourenne and Cleveland, 2009; Tamaoka et al., 2010). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.