The critical importance of p97 in cell and organismal physiology is underscored by the association of p97 mutations with neurodegenerative disorders including inclusion body myopathy with Paget’s disease of bone and frontotemporal dementia (IBMPFD) and amyotrophic lateral sclerosis (ALS)8. This evidence concerns the gene VCP and inclusion body myopathy with Paget disease of bone and frontotemporal dementia.