The RNA-binding proteins TDP-43 (TAR DNA-binding protein of 43 kDa) and FUS (Fused in sarcoma) have become infamous over the past years as being the main culprits in two fatal neurodegenerative diseases, ALS (amyotrophic lateral sclerosis) and FTD (frontotemporal dementia). This evidence concerns the gene TARDBP and frontotemporal dementia.