In recent years, mutations in several additional RNA-binding proteins have been associated with neurodegenerative diseases, including EWS (EWSR1), TAF15 (8), hnRNPA1, hnRNP A2B1 (9), and ataxin-2 (10), supporting the notion that defects in RNA metabolism can induce neurodegeneration (11, 12, 13). This evidence concerns the gene EWSR1 and neurodegenerative disease.