For example, in TDP-43 (TAR DNA-binding protein) single residue substitution mutation in the Gln/Asn-enriched LCR forms irreversible protein aggregates which are involved in amyotrophic lateral sclerosis and frontotemporal lobar dementia (Neumann et al., 2006; Johnson et al., 2009). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.