Valosin-containing protein (VCP), the homologue of yeast Cdc48, is the causative gene for a multisystem degenerative disease that was originally termed IBMPFD to encompass the wide range of debilitating clinical outcomes, including inclusion body myopathy (IBM), Paget's disease of the bone (PDB) and frontotemporal dementia (FD) (Watts et al., 2004). This evidence concerns the gene VCP and Fabry disease.