The critical role of VCP in cellular health is highlighted by the fact that genetic mutations in VCP have been associated with multiple neurodegenerative diseases including ALS,33 inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia,57 and Huntington’s disease.58 The precise mechanisms by which mutations trigger pathogenesis in the context of these diverse and devastating diseases remain unclear. Here, VCP is linked to frontotemporal dementia.