Importantly, many RBPs have been shown to have strong genetic links to neurodegenerative disease; for example, mutations in TDP-43, FUS, hnRNPA1, and ATXN2 have all been shown to cause amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration (FTLD) and/or spinocerebellar ataxia [24]. This evidence concerns the gene ATXN2 and amyotrophic lateral sclerosis.