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Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2018,5,22]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>Renal epithelial cells (RECs) contain abundant mitochondria that are essential to support renal reabsorption of electrolytes, glucose, and amino acids. However, it remains poorly understood how mitochondrial metabolism is coordinated with kidney reabsorptive functions. Here we show that deletion of estrogen-related receptor gamma (ERR\u03b3) in RECs results in severe renal mitochondrial and reabsorptive dysfunction with fluid-filled cysts. ERR\u03b3 directly regulates mitochondrial metabolism and cooperates in regulating renal reabsorption genes with hepatic nuclear factor 1 beta (HNF1\u03b2), mutations of which cause strikingly similar renal dysfunction and cysts in animals and humans. These findings reveal a role for ERR\u03b3 in simultaneously coordinating a transcriptional program of renal energy-generating mitochondrial and energy-consuming reabsorptive functions relevant to kidney disease.<\/jats:p>","DOI":"10.1073\/pnas.1804965115","type":"journal-article","created":{"date-parts":[[2018,5,22]],"date-time":"2018-05-22T13:05:35Z","timestamp":1526994335000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":44,"title":["Genomic integration of ERR\u03b3-HNF1\u03b2 regulates renal bioenergetics and prevents chronic kidney disease"],"prefix":"10.1073","volume":"115","author":[{"given":"Juanjuan","family":"Zhao","sequence":"first","affiliation":[{"name":"Center for Mitochondrial and Epigenomic Medicine, Children\u2019s Hospital of Philadelphia, Philadelphia, PA 19104;"},{"name":"Department of Pathology and Laboratory Medicine, Children\u2019s Hospital of Philadelphia, Philadelphia, PA 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Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104;"},{"name":"Department of Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian","family":"Liu","sequence":"additional","affiliation":[{"name":"Center for Mitochondrial and Epigenomic Medicine, Children\u2019s Hospital of Philadelphia, Philadelphia, PA 19104;"},{"name":"Department of Pathology and Laboratory Medicine, Children\u2019s Hospital of Philadelphia, Philadelphia, PA 19104;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuhiro","family":"Omura","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Endocrinology, Metabolism and Molecular Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL 60611"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda 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Diabetologia 42, 485\u2013492 (1999).","journal-title":"Diabetologia"},{"key":"e_1_3_4_3_2","doi-asserted-by":"crossref","first-page":"149","DOI":"10.5414\/CNP62149","article-title":"Progressive nephropathy associated with mitochondrial tRNA gene mutation","volume":"62","author":"Dinour D","year":"2004","unstructured":"D Dinour, S Mini, S Polak-Charcon, D Lotan, EJ Holtzman, Progressive nephropathy associated with mitochondrial tRNA gene mutation. Clin Nephrol 62, 149\u2013154 (2004).","journal-title":"Clin Nephrol"},{"key":"e_1_3_4_4_2","doi-asserted-by":"crossref","first-page":"F367","DOI":"10.1152\/ajprenal.00571.2013","article-title":"Mitochondrial dysfunction in the pathophysiology of renal diseases","volume":"306","author":"Che R","year":"2014","unstructured":"R Che, Y Yuan, S Huang, A Zhang, Mitochondrial dysfunction in the pathophysiology of renal diseases. 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