Interestingly, these suppressive effects of TDP-43, FUS, and hnRNPA2B1 differ from those of other RBPs that interact with expanded repeat RNAs such as MBNL1 in DM1 and hnRNPA3 in C9orf72-ALS/FTD (Figure 2). Here, HNRNPA2B1 is linked to frontotemporal dementia.