Human p97/VCP protein is part of the AAA+ ATPase protein family.1 p97 is ubiquitously expressed and is essential for a variety of cellular activities, including protein homeostasis, mitochondrial quality control, Golgi reassembly and autophagy.1,2 Mutations in p97 cause inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia (IBMPFD), (also known as multisystem proteinopathy 1, MSP1; OMIM 167320). Here, VCP is linked to inclusion body myopathy with Paget disease of bone and frontotemporal dementia.