The Nrf2 activator bardoxolone completed a phase 2 clinical trial for ADPKD, but the phase 3 trial was terminated early by the manufacturer due to insufficient efficacy at preventing end-stage kidney disease in another phase 3 trial using the same drug,21 leaving open the question whether Nrf2 activation is an effective strategy in ADPKD. Here, NFE2L2 is linked to autosomal dominant polycystic kidney disease.