RENAL PATHOPHYSIOLOGY: Edited by Orson W. Moe and Susan E. QuagginThe role of PPARα in autosomal dominant polycystic kidney diseaseLakhia, RonakAuthor Information Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA Correspondence to Ronak Lakhia, Division of Nephrology, Department of Internal Medicine, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, TX 75390, USA. Tel: +1 214 648 4576; fax: +1 214 645 6272; e-mail: email@example.com Current Opinion in Nephrology and Hypertension: July 2020 - Volume 29 - Issue 4 - p 432-438 doi: 10.1097/MNH.0000000000000615 Buy Metrics Abstract Purpose of review Metabolic reprogramming is a prominent feature of cyst epithelial cells in autosomal dominant polycystic kidney disease (ADPKD). Peroxisome proliferator activated receptor alpha (PPARα) is a transcription factor that regulates many aspects of cellular metabolism. The purpose of this review is to understand the role of PPARα in ADPKD. Recent findings PPARα expression is reduced in ADPKD kidneys of mice and humans. This downregulation is in part secondary to microRNA mediated translational repression and leads to impairment of fatty acid metabolism. Genetic studies demonstrate that deletion of Pparα aggravates cyst growth in a slowly progressive mouse model of ADPKD. Recent studies also show that administration of Pparα agonists ameliorates cyst burden in mice. Summary Abnormal reduction of PPARα affects cellular metabolism in ADPKD. Pparα is a modulator of cyst progression in mouse models of ADPKD. These studies establish PPARα as an exciting new drug target for the treatment of individuals with ADPKD. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.