Experimental models used to obtain these results comprised: autopsy brain samples, human primary skin fibroblasts, iPSCs-derived astrocytes, motor and cortical neurons of patients with C9ORF72- or TARDBP-linked ALS–FTSD, Drosophila and mouse models [161,162,163,164,165,166,167,168,169,170,171,172]. Here, TARDBP is linked to amyotrophic lateral sclerosis.