In humans, autosomal-dominant mutations in the VCP gene cause severe multisystem degenerative diseases such as inclusion body myopathy associated with Paget’s disease of the bone and frontotemporal dementia (IBMPFD), amyotrophic lateral sclerosis (ALS), Parkinson’s disease (PD), or myofibrillar myopathies (MFMs) [4,5,6,7,32]]. This evidence concerns the gene VCP and inclusion body myopathy with Paget disease of bone and frontotemporal dementia.