Single amino acid mutations in human p97 have been linked to diseases of aging and neurodegeneration, including IBMPFD (inclusion body myopathy with Paget’s disease of bone and frontotemporal dementia) and familial amyotrophic lateral sclerosis (ALS) [14,15,16,17]. The gene discussed is VCP; the disease is inclusion body myopathy with Paget disease of bone and frontotemporal dementia.