Genetic studies in the past decade have linked a collection of p97 mutations to human diseases including MSP1 (multisystem proteinopathy 1) [also named IBMPFD (Inclusion Body Myopathy associated with Paget's disease of the bone and Frontotemporal Dementia)], FALS (familial amyotrophic lateral sclerosis), CMT2Y (Charcot-Marie-Tooth disease, type 2Y) (Dyck and Lambert, 1968; Watts et al., 2004; Johnson et al., 2010; Abramzon et al., 2012; Bucelli et al., 2015), hereditary spastic paraplegias (HSP), Parkinson's disease (PD), and Alzheimer's disease (AD). Here, VCP is linked to frontotemporal dementia.