In the majority of sALS, as well as nearly half of frontotemporal dementia (FTD) cases, ubiquitinated and phosphorylated cytosolic TDP-43 aggregates are found in the frontal cortex (Neumann et al., 2006; Braak et al., 2010), whereas motor neurons of ALS patients harboring mutations in SOD1 or FUS display either neurofilamentous hyaline conglomerate inclusions and aggregates of misfolded SOD1 or cytoplasmic inclusions immunoreactive for FUS (Shibata et al., 1996; Kwiatkowski et al., 2009). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.