Furthermore, since p97 gain-of-function mutants have been proposed as a cause of pathogenesis in inclusion body myopathy, Paget’s disease of bone, and frontotemporal dementia (IBMPFD) [10,11,12], the inhibition of p97 activity may be exploited as a therapeutic strategy for IBMPFD. Here, VCP is linked to inclusion body myopathy with Paget disease of bone and frontotemporal dementia.