VCP and frontotemporal dementia: Missense mutations in the VCP gene, located on chromosome 9p13.3, were originally reported in 2004 in North American families with a specific autosomal dominant multisystem syndrome called inclusion body myopathy with Paget disease of bone and/or FTD (IBMPFD) (Watts et al., 2004), with neuropathology consistent with type D TDP-43 pathology (van der Zee et al., 2009).