Originally described as an oncogenic fusion to the CCAAT/enhancer-binding protein homologous protein (CHOP) transcription factor in myxoid liposarcoma (3, 4), FUS rose to prominence with the discovery that inherited, and de novo mutations in its ORF cause dominant forms of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) (5, 6, 7). The gene discussed is DDIT3; the disease is amyotrophic lateral sclerosis.