Pluto Bioinformatics

GSE104906: ERR coordinates a transcriptional program of mitochondrial and renal reabsorptive functions implicated in kidney disease[RNA-seq]

Bulk RNA sequencing

Here we show that estrogen-related receptor (ERR) is an essential transcriptional coordinator of both renal mitochondrial and reabsorptive functions. ERR is highly and specifically expressed in the RECs, and its expression correlates with kidney function in humans. We generated REC-ERR KO mice which developed severe renal mitochondrial and reabsorptive dysfunction and fluid-filled cysts. Transcriptome and cistrome analysis revealed that ERR directly regulates mitochondrial metabolism and renal reabsorption. ChIP-reChIP-Seq studies further suggest that ERR employs a distinct mechanism to regulate renal reabsorption genes through functional cooperation with hepatic nuclear factor 1 beta (HNF1), mutations of which cause strikingly similar renal dysfunction and cysts in humans and animals. Together these findings reveal a novel role for ERR in simultaneously coordinating a transcriptional program for renal energy production via mitochondria and energy consumption to perform reabsorptive functions required for normal kidney function SOURCE: Grant,D.,Baish Northwestern University