PPARα was shown to be downregulated in aggressive mouse models of autosomal dominant polycystic kidney disease (ADPKD) and primary human ADPKD cells, suggesting that decreased PPARα function may underlie the impaired FAO and oxidative phosphorylation in ADPKD (Hajarnis et al., 2017; Lakhia et al., 2018). The gene discussed is PPARA; the disease is autosomal dominant polycystic kidney disease.