Depletion of p97 is lethal, and point mutations in p97 cause a degenerative disease in humans that features inclusion body myopathy (IBM), fronto-temporal dementia (FTD), amyotrophic lateral sclerosis (ALS), and Paget’s disease (PD) of bone (collectively called IBMPFD/ALS) [12]. The gene discussed is VCP; the disease is amyotrophic lateral sclerosis.