Importantly, activation of Nrf1 not only increased the levels of many proteasome subunits, including 20S CP, 19S RP, and 11S RP components, it also increased the ubiquitination of polyQ-expanded AR and mediated its proteasomal degradation in SBMA patient fibroblasts and in Drosophila and mouse models of SBMA (Bott et al., 2016), indicating a therapeutic approach to enhance mutant AR degradation. Here, NRF1 is linked to Kennedy disease.