ArticleProceedings of the National Academy of Sciences of the United States of America2018
Genomic integration of ERRγ-HNF1β regulates renal bioenergetics and prevents chronic kidney disease.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 48 citations in OpenAlex.
- ESRRG downregulation in early spontaneous abortion induces mitochondrial damage, leading to impaired trophoblast function.Annals of medicine · 2026Article
- ERRγ deletion in podocytes accelerates aging related kidney disease.bioRxiv : the preprint server for biology · 2026Article
- Partial restoration of mitochondrial dysfunction by AAV-Ant1 protects from dilated cardiomyopathy in Ant1Nature communications · 2025Article
- The nonsteroidal MR antagonist finerenone reverses Western diet-induced kidney disease by regulating mitochondrial and lipid metabolism and inflammation.American journal of physiology. Renal physiology · 2025Article
- Genetics-nutrition interactions control diurnal enhancer-promoter dynamics and liver lipid metabolism.Cell metabolism · 2025Article
- Role of nuclear receptors, lipid metabolism, and mitochondrial function in the pathogenesis of diabetic kidney disease.American journal of physiology. Renal physiology · 2025Review
- Nuclear receptors in health and disease: signaling pathways, biological functions and pharmaceutical interventions.Signal transduction and targeted therapy · 2025Review
- Tails of nephron ciliated cell development: insights on patterning a functional tissue barrier from the zebrafish.Tissue barriers · 2024Review
- Epithelial cell states associated with kidney and allograft injury.Nature reviews. Nephrology · 2024Review
- Reprogramming of Energy Metabolism in HumanInternational journal of molecular sciences · 2024Article
- Single-cell multiomics guided mechanistic understanding of Fontan-associated liver disease.Science translational medicine · 2024Article
- Predicting proximal tubule failed repair drivers through regularized regression analysis of single cell multiomic sequencing.Nature communications · 2024Article
- Cardiac maturation.Journal of molecular and cellular cardiology · 2024Review
- Article
- Esrrγa regulates nephron and ciliary development by controlling prostaglandin synthesis.Development (Cambridge, England) · 2023Article
- Role of Estrogen-Related Receptor γ and PGC-1α/SIRT3 Pathway in Early Brain Injury After Subarachnoid Hemorrhage.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2023Article
- The multifaceted role of kidney tubule mitochondrial dysfunction in kidney disease development.Trends in cell biology · 2022Review
- Analysis of the Hypoxic Response in a Mouse Cortical Collecting Duct-Derived Cell Line Suggests That Esrra Is Partially Involved in Hif1α-Mediated Hypoxia-Inducible Gene Expression in mCCDInternational journal of molecular sciences · 2022Article
- The nuclear receptor ERR cooperates with the cardiogenic factor GATA4 to orchestrate cardiomyocyte maturation.Nature communications · 2022Article
- Sirtuin 5 depletion impairs mitochondrial function in human proximal tubular epithelial cells.Scientific reports · 2021Article
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Authors and funding
11 authors at 3 institutions in 1 country.
Funding
Abstract
Mitochondrial dysfunction is increasingly recognized as a critical determinant of both hereditary and acquired kidney diseases. However, it remains poorly understood how mitochondrial metabolism is regulated to support normal kidney function and how its dysregulation contributes to kidney disease. Here, we show that the nuclear receptor estrogen-related receptor gamma (ERRγ) and hepatocyte nuclear factor 1 beta (HNF1β) link renal mitochondrial and reabsorptive functions through coordinated epigenomic programs. ERRγ directly regulates mitochondrial metabolism but cooperatively controls renal reabsorption via convergent binding with HNF1β. Deletion of ERRγ in renal epithelial cells (RECs), in which it is highly and specifically expressed, results in severe renal energetic and reabsorptive dysfunction and progressive renal failure that recapitulates phenotypes of animals and patients with HNF1β loss-of-function gene mutations. Moreover, ERRγ expression positively correlates with renal function and is decreased in patients with chronic kidney disease (CKD). REC-ERRγ KO mice share highly overlapping renal transcriptional signatures with human patients with CKD. Together these findings reveal a role for ERRγ in directing independent and HNF1β-integrated programs for energy production and use essential for normal renal function and the prevention of kidney disease.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.