The p97/Cdc48 protein is essential for viability, but single site variants in human p97 have been linked to amyotrophic lateral sclerosis (ALS) [17] and to the multiple-disorder known as inclusion body myopathy associated with Paget's disease of the bone and frontotemporal dementia (IBMPFD) [18] [19]. Here, VCP is linked to inclusion body myopathy with Paget disease of bone and frontotemporal dementia.