First, we analysed if TDP-43, a PrLD-containing RBP shown to participate in SG formation [27] and heavily implicated in other neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS) [28, 29] and Alzheimer’s disease (AD) [30, 31], was affected by mutant ATXN7. Here, TARDBP is linked to amyotrophic lateral sclerosis.