Several RNA helicases facilitate splicing and alternative splicing of mRNAs (reviewed in (Bourgeois et al., 2016)) and dysregulation of splicing has been reported for microsatellite repeat expansion disorders, including DM, C9ORF72-ALS/FTD and several Spinocerebellar ataxias (reviewed in (Hale et al., 2019)). Here, DHX16 is linked to frontotemporal dementia.