Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are often referred to as ALS/FTD spectrum disorder.1 In FTD, fused in sarcoma (FUS), transactive response (TAR) DNA-binding protein 43 (TDP-43) and tau are pathological hallmarks.2 Valosin-containing protein (VCP/p97) is a ubiquitously expressed AAA+ ATPase with crucial roles in a multitude of intracellular functions3 and is directly implicated in both FTD and ALS. The gene discussed is RBM8A; the disease is frontotemporal dementia.