Tmic
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NameSteroid hormone receptor ERR1
Synonyms
  • ERR-alpha
  • ERR1
  • ESRL1
  • Estrogen receptor-like 1
  • Estrogen-related receptor alpha
  • NR3B1
  • Nuclear receptor subfamily 3 group B member 1
Gene NameESRRA
OrganismHuman
Amino acid sequence
>lcl|BSEQ0007149|Steroid hormone receptor ERR1
MSSQVVGIEPLYIKAEPASPDSPKGSSETETEPPVALAPGPAPTRCLPGHKEEEDGEGAG
PGEQGGGKLVLSSLPKRLCLVCGDVASGYHYGVASCEACKAFFKRTIQGSIEYSCPASNE
CEITKRRRKACQACRFTKCLRVGMLKEGVRLDRVRGGRQKYKRRPEVDPLPFPGPFPAGP
LAVAGGPRKTAAPVNALVSHLLVVEPEKLYAMPDPAGPDGHLPAVATLCDLFDREIVVTI
SWAKSIPGFSSLSLSDQMSVLQSVWMEVLVLGVAQRSLPLQDELAFAEDLVLDEEGARAA
GLGELGAALLQLVRRLQALRLEREEYVLLKALALANSDSVHIEDAEAVEQLREALHEALL
EYEAGRAGPGGGAERRRAGRLLLTLPLLRQTAGKVLAHFYGVKLEGKVPMHKLFLEMLEA
MMD
Number of residues423
Molecular Weight45509.11
Theoretical pI6.33
GO Classification
Functions
  • protein domain specific binding
  • sequence-specific DNA binding
  • RNA polymerase II transcription factor activity, ligand-activated sequence-specific DNA binding
  • RNA polymerase II core promoter proximal region sequence-specific DNA binding
  • transcriptional activator activity, RNA polymerase II core promoter proximal region sequence-specific binding
  • zinc ion binding
  • steroid hormone receptor activity
  • DNA binding
  • transcriptional repressor activity, RNA polymerase II core promoter proximal region sequence-specific binding
  • steroid binding
Processes
  • intracellular receptor signaling pathway
  • gene expression
  • cartilage development
  • transcription initiation from RNA polymerase II promoter
  • regulation of osteoblast differentiation
  • regulation of cell proliferation
  • regulation of osteoclast differentiation
  • regulation of transcription, DNA-templated
  • positive regulation of transcription from RNA polymerase II promoter
  • organelle organization
  • mitochondrion organization
Components
  • nucleolus
  • nucleoplasm
  • microtubule cytoskeleton
  • intercellular bridge
  • nucleus
General FunctionZinc ion binding
Specific FunctionBinds to an ERR-alpha response element (ERRE) containing a single consensus half-site, 5'-TNAAGGTCA-3'. Can bind to the medium-chain acyl coenzyme A dehydrogenase (MCAD) response element NRRE-1 and may act as an important regulator of MCAD promoter. Binds to the C1 region of the lactoferrin gene promoter. Requires dimerization and the coactivator, PGC-1A, for full activity. The ERRalpha/PGC1alpha complex is a regulator of energy metabolism. Induces the expression of PERM1 in the skeletal muscle.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID36609
UniProtKB IDP11474
UniProtKB Entry NameERR1_HUMAN
Cellular LocationNucleus
Gene sequence
>lcl|BSEQ0012595|Steroid hormone receptor ERR1 (ESRRA)
ATGTCCAGCCAGGTGGTGGGCATTGAGCCTCTCTACATCAAGGCAGAGCCGGCCAGCCCT
GACAGTCCAAAGGGTTCCTCGGAGACAGAGACCGAGCCTCCTGTGGCCCTGGCCCCTGGT
CCAGCTCCCACTCGCTGCCTCCCAGGCCACAAGGAAGAGGAGGATGGGGAGGGGGCTGGG
CCTGGCGAGCAGGGCGGTGGGAAGCTGGTGCTCAGCTCCCTGCCCAAGCGCCTCTGCCTG
GTCTGTGGGGACGTGGCCTCCGGCTACCACTATGGTGTGGCATCCTGTGAGGCCTGCAAA
GCCTTCTTCAAGAGGACCATCCAGGGGAGCATCGAGTACAGCTGTCCGGCCTCCAACGAG
TGTGAGATCACCAAGCGGAGACGCAAGGCCTGCCAGGCCTGCCGCTTCACCAAGTGCCTG
CGGGTGGGCATGCTCAAGGAGGGAGTGCGCCTGGACCGCGTCCGGGGTGGGCGGCAGAAG
TACAAGCGGCGGCCGGAGGTGGACCCACTGCCCTTCCCGGGCCCCTTCCCTGCTGGGCCC
CTGGCAGTCGCTGGAGGCCCCCGGAAGACAGCAGCCCCAGTGAATGCACTGGTGTCTCAT
CTGCTGGTGGTTGAGCCTGAGAAGCTCTATGCCATGCCTGACCCCGCAGGCCCTGATGGG
CACCTCCCAGCCGTGGCTACCCTCTGTGACCTCTTTGACCGAGAGATTGTGGTCACCATC
AGCTGGGCCAAGAGCATCCCAGGCTTCTCATCGCTGTCGCTGTCTGACCAGATGTCAGTA
CTGCAGAGCGTGTGGATGGAGGTGCTGGTGCTGGGTGTGGCCCAGCGCTCACTGCCACTG
CAGGATGAGCTGGCCTTCGCTGAGGACTTAGTCCTGGATGAAGAGGGGGCACGGGCAGCT
GGCCTGGGGGAACTGGGGGCTGCCCTGCTGCAACTAGTGCGGCGGCTGCAGGCCCTGCGG
CTGGAGCGAGAGGAGTATGTTCTACTAAAGGCCTTGGCCCTTGCCAATTCAGACTCTGTG
CACATCGAAGATGCCGAGGCTGTGGAGCAGCTGCGAGAAGCTCTGCACGAGGCCCTGCTG
GAGTATGAAGCCGGCCGGGCTGGCCCCGGAGGGGGTGCTGAGCGGCGGCGGGCGGGCAGG
CTGCTGCTCACGCTACCGCTCCTCCGCCAGACAGCGGGCAAAGTGCTGGCCCATTTCTAT
GGGGTGAAGCTGGAGGGCAAGGTGCCCATGCACAAGCTGTTCTTGGAGATGCTCGAGGCC
ATGATGGACTGA
GenBank Gene IDX51416
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:3471
Chromosome Location11
Locus11q13
References
  1. Giguere V, Yang N, Segui P, Evans RM: Identification of a new class of steroid hormone receptors. Nature. 1988 Jan 7;331(6151):91-4. 3267207
  2. Yang N, Shigeta H, Shi H, Teng CT: Estrogen-related receptor, hERR1, modulates estrogen receptor-mediated response of human lactoferrin gene promoter. J Biol Chem. 1996 Mar 8;271(10):5795-804. 8621448
  3. Taylor TD, Noguchi H, Totoki Y, Toyoda A, Kuroki Y, Dewar K, Lloyd C, Itoh T, Takeda T, Kim DW, She X, Barlow KF, Bloom T, Bruford E, Chang JL, Cuomo CA, Eichler E, FitzGerald MG, Jaffe DB, LaButti K, Nicol R, Park HS, Seaman C, Sougnez C, Yang X, Zimmer AR, Zody MC, Birren BW, Nusbaum C, Fujiyama A, Hattori M, Rogers J, Lander ES, Sakaki Y: Human chromosome 11 DNA sequence and analysis including novel gene identification. Nature. 2006 Mar 23;440(7083):497-500. 16554811
  4. Wiley SR, Kraus RJ, Zuo F, Murray EE, Loritz K, Mertz JE: SV40 early-to-late switch involves titration of cellular transcriptional repressors. Genes Dev. 1993 Nov;7(11):2206-19. 8224847
  5. Sladek R, Bader JA, Giguere V: The orphan nuclear receptor estrogen-related receptor alpha is a transcriptional regulator of the human medium-chain acyl coenzyme A dehydrogenase gene. Mol Cell Biol. 1997 Sep;17(9):5400-9. 9271417
  6. Schreiber SN, Knutti D, Brogli K, Uhlmann T, Kralli A: The transcriptional coactivator PGC-1 regulates the expression and activity of the orphan nuclear receptor estrogen-related receptor alpha (ERRalpha). J Biol Chem. 2003 Mar 14;278(11):9013-8. Epub 2003 Jan 8. 12522104
  7. Barry JB, Laganiere J, Giguere V: A single nucleotide in an estrogen-related receptor alpha site can dictate mode of binding and peroxisome proliferator-activated receptor gamma coactivator 1alpha activation of target promoters. Mol Endocrinol. 2006 Feb;20(2):302-10. Epub 2005 Sep 8. 16150865
  8. Vu EH, Kraus RJ, Mertz JE: Phosphorylation-dependent sumoylation of estrogen-related receptor alpha1. Biochemistry. 2007 Aug 28;46(34):9795-804. Epub 2007 Aug 4. 17676930
  9. Tremblay AM, Wilson BJ, Yang XJ, Giguere V: Phosphorylation-dependent sumoylation regulates estrogen-related receptor-alpha and -gamma transcriptional activity through a synergy control motif. Mol Endocrinol. 2008 Mar;22(3):570-84. Epub 2007 Dec 6. 18063693
  10. Dephoure N, Zhou C, Villen J, Beausoleil SA, Bakalarski CE, Elledge SJ, Gygi SP: A quantitative atlas of mitotic phosphorylation. Proc Natl Acad Sci U S A. 2008 Aug 5;105(31):10762-7. doi: 10.1073/pnas.0805139105. Epub 2008 Jul 31. 18669648
  11. 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. 20484414
  12. 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. 23836911
  13. Kallen J, Schlaeppi JM, Bitsch F, Filipuzzi I, Schilb A, Riou V, Graham A, Strauss A, Geiser M, Fournier B: Evidence for ligand-independent transcriptional activation of the human estrogen-related receptor alpha (ERRalpha): crystal structure of ERRalpha ligand binding domain in complex with peroxisome proliferator-activated receptor coactivator-1alpha. J Biol Chem. 2004 Nov 19;279(47):49330-7. Epub 2004 Aug 26. 15337744
  14. Greschik H, Althage M, Flaig R, Sato Y, Chavant V, Peluso-Iltis C, Choulier L, Cronet P, Rochel N, Schule R, Stromstedt PE, Moras D: Communication between the ERRalpha homodimer interface and the PGC-1alpha binding surface via the helix 8-9 loop. J Biol Chem. 2008 Jul 18;283(29):20220-30. doi: 10.1074/jbc.M801920200. Epub 2008 Apr 25. 18441008