Aberrant activity and localization of two RBPs, TDP-43 and FUS RNA binding protein (FUS), are implicated as the pathogenicity of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), two fatal neurodegenerative disorders that share clinical, genetic, and pathologic hallmarks [131]. The gene discussed is FUS; the disease is frontotemporal dementia.