Pathogenic VCP/p97 mutations cause a multisystem proteinopathy linked to different pathologies, such as inclusion bodies myopathy associated with Paget’s disease of the bone and FTD (IBMPFD) as well as ALS (Watts et al., 2004; Kimonis et al., 2008; Johnson et al., 2010; Gonzalez-Perez et al., 2012; Gang et al., 2016). Here, VCP is linked to inclusion body myopathy with Paget disease of bone and frontotemporal dementia.