An increasing number of the ALS/FTD-associated RBPs, including FUS, hnRNPA1, hnRNPA2B1, TIA1, Matrin 3, and Ataxin 2, are found as SG components7,9–12; moreover, several ALS/FTD-associated non-RBPs, including dipeptide repeats (C9-DPRs) derived from C9orf72 hexanucleotide repeat expansions, SOD1, p62, UBQLN2, and VCP, are found to co-localize with SGs and regulate their dynamics13–17. This evidence concerns the gene ATXN2 and frontotemporal dementia.