This is reminiscent of our recent study on SCA31, in which we demonstrated a novel role of the ALS/FTD-linked RBPs TDP-43, FUS, and hnRNPA2B1 as RNA chaperones binding to UGGAA repeat RNA and altering its structure, resulting in the suppression of its neurotoxicity through reducing RNA foci formation and repeat polypeptide translation (Ishiguro et al., 2017). This evidence concerns the gene FUS and frontotemporal dementia.