In fact, VCP mutations were reported in a wide range of disorders, including frontotemporal dementia (FTD), Charcot–Marie–Tooth disease, inclusion body myopathy, Paget disease of bone, Parkinson’s disease and amyotrophic lateral sclerosis (ALS).8 While cytoplasmic accumulation and nuclear loss of TDP-43 in motor neurons are considered a hallmark of the ALS disease at late stages,9 abnormal alternative splicing is an earlier sign of the disease,10 which implies RNAPII accessory proteins are probably involved in the onset of the disease. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.