Since many characterized ALS-causing genes encode RNA-binding proteins (RBPs)—such as angiogenin (ANG), TAR DNA-binding protein 43 (TDP-43), FUS, Ataxin-2 (ATXN2), chromosome 9 open reading frame 72 (C9ORF72), TATA-box binding protein associated factor 15 (TAF15) and heterogeneous nuclear ribonucleoprotein A1 (HNRNPA1)—it is not surprising that global and/or selective RBP-RNAs, including lncRNAs, might participate in ALS onset or disease progression. This evidence concerns the gene ATXN2 and amyotrophic lateral sclerosis.