Single missense mutations in the VCP gene are the cause of frontotemporal dementia (IBMPFD) (Kimonis et al., 2000; Watts et al., 2004) and may account for 1%–2% of familial amyotrophic lateral sclerosis (ALS) (Johnson et al., 2010). This evidence concerns the gene VCP and familial amyotrophic lateral sclerosis.