Steroid hormone receptor ERR2

Details

Name
Steroid hormone receptor ERR2
Kind
protein
Synonyms
  • ERR beta-2
  • ERR-beta
  • ERRB2
  • ESRL2
  • Estrogen receptor-like 2
  • Estrogen-related receptor beta
  • NR3B2
  • Nuclear receptor subfamily 3 group B member 2
Gene Name
ESRRB
UniProtKB Entry
O95718Swiss-Prot
Organism
Humans
NCBI Taxonomy ID
9606
Amino acid sequence
>lcl|BSEQ0055588|Steroid hormone receptor ERR2
MSSDDRHLGSSCGSFIKTEPSSPSSGIDALSHHSPSGSSDASGGFGLALGTHANGLDSPP
MFAGAGLGGTPCRKSYEDCASGIMEDSAIKCEYMLNAIPKRLCLVCGDIASGYHYGVASC
EACKAFFKRTIQGNIEYSCPATNECEITKRRRKSCQACRFMKCLKVGMLKEGVRLDRVRG
GRQKYKRRLDSESSPYLSLQISPPAKKPLTKIVSYLLVAEPDKLYAMPPPGMPEGDIKAL
TTLCDLADRELVVIIGWAKHIPGFSSLSLGDQMSLLQSAWMEILILGIVYRSLPYDDKLV
YAEDYIMDEEHSRLAGLLELYRAILQLVRRYKKLKVEKEEFVTLKALALANSDSMYIEDL
EAVQKLQDLLHEALQDYELSQRHEEPWRTGKLLLTLPLLRQTAAKAVQHFYSVKLQGKVP
MHKLFLEMLEAKV
Number of residues
433
Molecular Weight
48053.14
Theoretical pI
Not Available
GO Classification
Functions
cis-regulatory region sequence-specific DNA binding / DNA-binding transcription activator activity, RNA polymerase II-specific / DNA-binding transcription factor activity / DNA-binding transcription factor activity, RNA polymerase II-specific / estrogen response element binding / nuclear receptor activity / nuclear steroid receptor activity / RNA polymerase II cis-regulatory region sequence-specific DNA binding / RNA polymerase II complex binding / RNA polymerase II-specific DNA-binding transcription factor binding / sequence-specific double-stranded DNA binding
Processes
cell dedifferentiation / cell population proliferation / inner ear development / negative regulation of stem cell differentiation / nuclear receptor-mediated steroid hormone signaling pathway / photoreceptor cell maintenance / positive regulation of DNA-templated transcription / positive regulation of stem cell population maintenance / positive regulation of transcription by RNA polymerase II / regulation of DNA-templated transcription / regulation of stem cell division / regulation of transcription by RNA polymerase II / stem cell division
Components
chromatin / condensed chromosome / cytoplasm / cytosol
General Function
Transcription factor that binds a canonical ESRRB recognition (ERRE) sequence 5'TCAAGGTCA-3' localized on promoter and enhancer of targets genes regulating their expression or their transcription activity (PubMed:17920186, PubMed:19755138). Plays a role, in a LIF-independent manner, in maintainance of self-renewal and pluripotency of embryonic and trophoblast stem cells through different signaling pathways including FGF signaling pathway and Wnt signaling pathways. Upon FGF signaling pathway activation, interacts with KDM1A by directly binding to enhancer site of ELF5 and EOMES and activating their transcription leading to self-renewal of trophoblast stem cells. Also regulates expression of multiple rod-specific genes and is required for survival of this cell type (By similarity). Plays a role as transcription factor activator of GATA6, NR0B1, POU5F1 and PERM1 (PubMed:23836911). Plays a role as transcription factor repressor of NFE2L2 transcriptional activity and ESR1 transcriptional activity (PubMed:17920186, PubMed:19755138). During mitosis remains bound to a subset of interphase target genes, including pluripotency regulators, through the canonical ESRRB recognition (ERRE) sequence, leading to their transcriptional activation in early G1 phase. Can coassemble on structured DNA elements with other transcription factors like SOX2, POU5F1, KDM1A and NCOA3 to trigger ESRRB-dependent gene activation. This mechanism, in the case of SOX2 corecruitment prevents the embryonic stem cells (ESCs) to epiblast stem cells (EpiSC) transition through positive regulation of NR0B1 that inhibits the EpiSC transcriptional program. Also plays a role inner ear development by controlling expression of ion channels and transporters and in early placentation (By similarity)
Specific Function
cis-regulatory region sequence-specific DNA binding
Pfam Domain Function
Signal Regions
Not Available
Transmembrane Regions
Not Available
Cellular Location
Nucleus
Gene sequence
>lcl|BSEQ0021406|Steroid hormone receptor ERR2 (ESRRB)
ATGTCCTCGGACGACAGGCACCTGGGCTCCAGCTGCGGCTCCTTCATCAAGACTGAGCCG
TCCAGCCCGTCCTCGGGCATCGATGCCCTCAGCCACCACAGCCCCAGTGGCTCGTCCGAC
GCCAGCGGCGGCTTTGGCCTGGCCCTGGGCACCCACGCCAACGGTCTGGACTCGCCACCC
ATGTTTGCAGGCGCCGGGCTGGGAGGCACCCCATGCCGCAAGAGCTACGAGGACTGTGCC
AGCGGCATCATGGAGGACTCGGCCATCAAGTGCGAGTACATGCTCAACGCCATCCCCAAG
CGCCTGTGCCTCGTGTGCGGGGACATTGCCTCTGGCTACCACTACGGCGTGGCCTCCTGC
GAGGCTTGCAAGGCCTTCTTCAAGAGGACTATCCAAGGGAACATTGAGTACAGCTGCCCG
GCCACCAACGAGTGCGAGATCACCAAACGGAGGCGCAAGTCCTGCCAGGCCTGCCGCTTC
ATGAAATGCCTCAAAGTGGGGATGCTGAAGGAAGGTGTGCGCCTTGATCGAGTGCGTGGA
GGCCGTCAGAAATACAAGCGACGGCTGGACTCAGAGAGCAGCCCATACCTGAGCTTACAA
ATTTCTCCACCTGCTAAAAAGCCATTGACCAAGATTGTCTCATACCTACTGGTGGCTGAG
CCGGACAAGCTCTATGCCATGCCTCCCCCTGGTATGCCTGAGGGGGACATCAAGGCCCTG
ACCACTCTCTGTGACCTGGCAGACCGAGAGCTTGTGGTCATCATTGGCTGGGCCAAGCAC
ATCCCAGGCTTCTCAAGCCTCTCCCTGGGGGACCAGATGAGCCTGCTGCAGAGTGCCTGG
ATGGAAATCCTCATCCTGGGCATCGTGTACCGCTCGCTGCCCTATGACGACAAGCTGGTG
TACGCTGAGGACTACATCATGGATGAGGAGCACTCCCGCCTCGCGGGGCTGCTGGAGCTC
TACCGGGCCATCCTGCAGCTGGTACGCAGGTACAAGAAGCTCAAGGTGGAGAAGGAGGAG
TTTGTGACGCTCAAGGCCCTGGCCCTCGCCAACTCCGATTCCATGTACATCGAGGATCTA
GAGGCTGTCCAGAAGCTGCAGGACCTGCTGCACGAGGCACTGCAGGACTACGAGCTGAGC
CAGCGCCATGAGGAGCCCTGGAGGACGGGCAAGCTGCTGCTGACACTGCCGCTGCTGCGG
CAGACGGCCGCCAAGGCCGTGCAGCACTTCTATAGCGTCAAACTGCAGGGCAAAGTGCCC
ATGCACAAACTCTTCCTGGAGATGCTGGAGGCCAAGGTTGGCCAAGAGCAGCTTAGAGGA
TCTCCCAAGGATGAAAGAATGTCAAGCCATGATGGAAAATGCCCCTTCCAATCAGCTGCC
TTCACAAGCAGGGATCAGAGCAACTCCCCGGGGATCCCCAATCCACGCCCTTCTAGTCCA
ACCCCCCTCAATGAGAGAGGCAGGCAGATCTCACCCAGCACTAGGACACCAGGAGGCCAG
GGAAAGCATCTCTGGCTCACCATGTAA
Chromosome Location
14
Locus
14q24.3
External Identifiers
ResourceLink
UniProtKB IDO95718
UniProtKB Entry NameERR2_HUMAN
GeneCard IDESRRB
HGNC IDHGNC:3473
PDB ID(s)1LO1, 6LIT, 6LN4
KEGG IDhsa:2103
IUPHAR/Guide To Pharmacology ID623
NCBI Gene ID2103
General References
  1. Chen F, Zhang Q, McDonald T, Davidoff MJ, Bailey W, Bai C, Liu Q, Caskey CT: Identification of two hERR2-related novel nuclear receptors utilizing bioinformatics and inverse PCR. Gene. 1999 Mar 4;228(1-2):101-9. [Article]
  2. Zhou W, Liu Z, Wu J, Liu JH, Hyder SM, Antoniou E, Lubahn DB: Identification and characterization of two novel splicing isoforms of human estrogen-related receptor beta. J Clin Endocrinol Metab. 2006 Feb;91(2):569-79. Epub 2005 Dec 6. [Article]
  3. Heilig R, Eckenberg R, Petit JL, Fonknechten N, Da Silva C, Cattolico L, Levy M, Barbe V, de Berardinis V, Ureta-Vidal A, Pelletier E, Vico V, Anthouard V, Rowen L, Madan A, Qin S, Sun H, Du H, Pepin K, Artiguenave F, Robert C, Cruaud C, Bruls T, Jaillon O, Friedlander L, Samson G, Brottier P, Cure S, Segurens B, Aniere F, Samain S, Crespeau H, Abbasi N, Aiach N, Boscus D, Dickhoff R, Dors M, Dubois I, Friedman C, Gouyvenoux M, James R, Madan A, Mairey-Estrada B, Mangenot S, Martins N, Menard M, Oztas S, Ratcliffe A, Shaffer T, Trask B, Vacherie B, Bellemere C, Belser C, Besnard-Gonnet M, Bartol-Mavel D, Boutard M, Briez-Silla S, Combette S, Dufosse-Laurent V, Ferron C, Lechaplais C, Louesse C, Muselet D, Magdelenat G, Pateau E, Petit E, Sirvain-Trukniewicz P, Trybou A, Vega-Czarny N, Bataille E, Bluet E, Bordelais I, Dubois M, Dumont C, Guerin T, Haffray S, Hammadi R, Muanga J, Pellouin V, Robert D, Wunderle E, Gauguet G, Roy A, Sainte-Marthe L, Verdier J, Verdier-Discala C, Hillier L, Fulton L, McPherson J, Matsuda F, Wilson R, Scarpelli C, Gyapay G, Wincker P, Saurin W, Quetier F, Waterston R, Hood L, Weissenbach J: The DNA sequence and analysis of human chromosome 14. Nature. 2003 Feb 6;421(6923):601-7. Epub 2003 Jan 1. [Article]
  4. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [Article]
  5. Bombail V, Collins F, Brown P, Saunders PT: Modulation of ER alpha transcriptional activity by the orphan nuclear receptor ERR beta and evidence for differential effects of long- and short-form splice variants. Mol Cell Endocrinol. 2010 Jan 15;314(1):53-61. doi: 10.1016/j.mce.2009.09.007. Epub 2009 Sep 13. [Article]
  6. Cho Y, Hazen BC, Russell AP, Kralli A: Peroxisome proliferator-activated receptor gamma coactivator 1 (PGC-1)- and estrogen-related receptor (ERR)-induced regulator in muscle 1 (Perm1) is a tissue-specific regulator of oxidative capacity in skeletal muscle cells. J Biol Chem. 2013 Aug 30;288(35):25207-18. doi: 10.1074/jbc.M113.489674. Epub 2013 Jul 8. [Article]
  7. Gearhart MD, Holmbeck SM, Evans RM, Dyson HJ, Wright PE: Monomeric complex of human orphan estrogen related receptor-2 with DNA: a pseudo-dimer interface mediates extended half-site recognition. J Mol Biol. 2003 Apr 4;327(4):819-32. [Article]
  8. Bombail V, MacPherson S, Critchley HO, Saunders PT: Estrogen receptor related beta is expressed in human endometrium throughout the normal menstrual cycle. Hum Reprod. 2008 Dec;23(12):2782-90. doi: 10.1093/humrep/den298. Epub 2008 Sep 4. [Article]
  9. Wilson BJ, Tremblay AM, Deblois G, Sylvain-Drolet G, Giguere V: An acetylation switch modulates the transcriptional activity of estrogen-related receptor alpha. Mol Endocrinol. 2010 Jul;24(7):1349-58. doi: 10.1210/me.2009-0441. Epub 2010 May 19. [Article]
  10. Collin RW, Kalay E, Tariq M, Peters T, van der Zwaag B, Venselaar H, Oostrik J, Lee K, Ahmed ZM, Caylan R, Li Y, Spierenburg HA, Eyupoglu E, Heister A, Riazuddin S, Bahat E, Ansar M, Arslan S, Wollnik B, Brunner HG, Cremers CW, Karaguzel A, Ahmad W, Cremers FP, Vriend G, Friedman TB, Riazuddin S, Leal SM, Kremer H: Mutations of ESRRB encoding estrogen-related receptor beta cause autosomal-recessive nonsyndromic hearing impairment DFNB35. Am J Hum Genet. 2008 Jan;82(1):125-38. doi: 10.1016/j.ajhg.2007.09.008. [Article]

Associated Data

Drug Relations
DrugDrug groupPharmacological action?TypeActionsDetails
Diethylstilbestrolapproved, investigational, withdrawnunknowntargetDetails
Flavoneapproved, experimentalunknowntargetagonistDetails
GenisteininvestigationalunknowntargetagonistDetails
UlobetasolapprovedyestargetmodulatorDetails
Desonideapproved, investigationalyestargetmodulatorDetails
Fluocinonideapproved, investigationalyestargetmodulatorDetails
MedrysoneapprovedyestargetmodulatorDetails
Halcinonideapproved, investigational, withdrawnyestargetmodulatorDetails
AmcinonideapprovedyestargetmodulatorDetails
Prednicarbateapproved, investigationalyestargetmodulatorDetails
DesoximetasoneapprovedyestargetmodulatorDetails
Flumethasoneapproved, vet_approvedyestargetmodulatorDetails
Auranofinapproved, investigationalyestargetmodulatorDetails
Cortisone acetateapproved, investigationalyestargetmodulatorDetails
Loteprednol etabonateapprovedyestargetmodulatorDetails
FlurandrenolideapprovedyestargetmodulatorDetails