P62 gene encodes a multifunctional protein that binds ubiquitin and regulates activation of the nuclear factor kappa-B (NF-kB) signaling pathway. The protein functions as a scaffolding/adaptor protein in concert with TNF receptor-associated factor 6 to mediate activation of NF-kB in response to upstream signals. Mutations in the ubiquitin-associated (UBA) domain of the Sequestosome 1 protein (SQSTM1), also designated p62 or ZIP, commonly cause Paget’s disease since the UBA is necessary for aggregate sequestration and cell survival.
