Several human RBPs with PrLDs including ataxin 1, ataxin 2, TDP-43, FUS, TAF15, EWSR1, hnRNPA1, and hnRNPA2 (Table S1) feature prominently in the pathology and genetics of a number of fatal neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), and spinocereballar ataxias (Couthouis et al., 2011, 2012, 2014; Cushman et al., 2010; Elden et al., 2010; Kim et al., 2013; Kwiatkowski et al., 2009; Neumann et al., 2006; Orr and Zoghbi, 2007; Orr, 2012; Vance et al., 2009; Zoghbi and Orr, 2009). This evidence concerns the gene ATXN1 and frontotemporal dementia.