The inclusion bodies, which are not unique to Paget’s disease, but probably relevant to its pathogenesis, are now thought to be aggregates containing ubiquitin and RNA-binding proteins, such as, hnRNPA1, and hnRNPA2B1, but may also include proteins that mediate ubiquitin-dependent autophagy, including p62/SQSTM1, and VCP amongst others, that form because of defects in the cellular degradation machinery [1, 24]. The gene discussed is SQSTM1; the disease is Paget disease.