The role of PPARαin autosomal dominant polycystic kidney disease

Ronak Lakhia

doi:10.1097/MNH.0000000000000615

The role of PPAR_αin autosomal dominant polycystic kidney disease

Ronak Lakhia

Research output: Contribution to journal › Review article › peer-review

7 Scopus citations

Abstract

Purpose of reviewMetabolic 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 findingsPPARα 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.SummaryAbnormal 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.

Original language	English (US)
Pages (from-to)	432-438
Number of pages	7
Journal	Current opinion in nephrology and hypertension
Volume	29
Issue number	4
DOIs	https://doi.org/10.1097/MNH.0000000000000615
State	Published - Jul 1 2020

Keywords

ADPKD
PPARα
metabolism

ASJC Scopus subject areas

Internal Medicine
Nephrology

Access to Document

10.1097/MNH.0000000000000615

Cite this

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title = "The role of PPARαin autosomal dominant polycystic kidney disease",

abstract = "Purpose of reviewMetabolic 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 findingsPPARα 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.SummaryAbnormal 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.",

keywords = "ADPKD, PPARα, metabolism",

author = "Ronak Lakhia",

note = "Funding Information: The work from the author{\textquoteright}s laboratory is supported by National Institute of Health (K08DK117049) and the University of Texas Southwestern Medical Center O{\textquoteright}Brien Kidney Research Core Center (NIDDK, P30DK079328). Publisher Copyright: {\textcopyright} 2020 Lippincott Williams and Wilkins. All rights reserved.",

year = "2020",

month = jul,

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doi = "10.1097/MNH.0000000000000615",

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N1 - Funding Information: The work from the author’s laboratory is supported by National Institute of Health (K08DK117049) and the University of Texas Southwestern Medical Center O’Brien Kidney Research Core Center (NIDDK, P30DK079328). Publisher Copyright: © 2020 Lippincott Williams and Wilkins. All rights reserved.

PY - 2020/7/1

Y1 - 2020/7/1

N2 - Purpose of reviewMetabolic 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 findingsPPARα 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.SummaryAbnormal 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.

AB - Purpose of reviewMetabolic 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 findingsPPARα 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.SummaryAbnormal 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.

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KW - metabolism

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