The role of PPARα in autosomal dominant polycystic kidney disease.

Ronak Lakhia

Current opinion in nephrology and hypertension 2020 Jul

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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. 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. 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.

Citation

Ronak Lakhia. The role of PPARα in autosomal dominant polycystic kidney disease. Current opinion in nephrology and hypertension. 2020 Jul;29(4):432-438