Although several other RNA binding (e.g., senataxin, hnRNPA1 Chen et al., 2004; Kim et al., 2013) or stress granule associated proteins (e.g., TIA-1, profilin Liu-Yesucevitz et al., 2010; Wu et al., 2012; Figley et al., 2014) have been linked to ALS/FTD, here we will address some of these questions by reviewing recent literature on RNA metabolism in ALS and discuss how dysregulation of RNA processing steps may contribute to disease from the perspective of three critically important genes, namely TARDP, FUS and C9orf72 (Figure 1). Here, FUS is linked to amyotrophic lateral sclerosis.