Abnormal aggregate formation of some RBPs (like TDP-43 and FUS) or structural proteins (like Tau and α-Synuclein) are characteristics of neurodegenerative diseases like frontal temporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) for the former two (Neumann et al., 2006; Cairns et al., 2007; Vance et al., 2009; Mackenzie et al., 2010), and familial Alzheimer’s and Parkinson’s diseases for the latter two (Alonso et al., 1996; Polymeropoulos et al., 1997). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.