Gene/Proteome Database (LMPD)

LMPD ID
LMP001124
Gene ID
2101
Species
Homo sapiens (Human)
Gene Name
estrogen-related receptor alpha
Gene Symbol
ESRRA
Synonyms
ERR1; ERRa; ERRalpha; ESRL1; NR3B1
Alternate Names
steroid hormone receptor ERR1; ERR-alpha; estrogen receptor-like 1; estrogen-related nuclear receptor alpha; nuclear receptor subfamily 3 group B member 1
Chromosome
11
Map Location
11q13
Summary
The protein encoded by this gene is a nuclear receptor that is closely related to the estrogen receptor. This protein acts as a site-specific transcription regulator and has been also shown to interact with estrogen and the transcripton factor TFIIB by direct protein-protein contact. The binding and regulatory activities of this protein have been demonstrated in the regulation of a variety of genes including lactoferrin, osteopontin, medium-chain acyl coenzyme A dehydrogenase (MCAD) and thyroid hormone receptor genes. A processed pseudogene of ESRRA is located on chromosome 13q12.1. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Sep 2013]
Orthologs
View orthologs and multiple alignments for ESRRA

Proteins

steroid hormone receptor ERR1 isoform 1
Refseq ID NP_004442
Protein GI 18860920
UniProt ID P11474
mRNA ID NM_004451
Length 423
RefSeq Status REVIEWED
MSSQVVGIEPLYIKAEPASPDSPKGSSETETEPPVALAPGPAPTRCLPGHKEEEDGEGAGPGEQGGGKLVLSSLPKRLCLVCGDVASGYHYGVASCEACKAFFKRTIQGSIEYSCPASNECEITKRRRKACQACRFTKCLRVGMLKEGVRLDRVRGGRQKYKRRPEVDPLPFPGPFPAGPLAVAGGPRKTAAPVNALVSHLLVVEPEKLYAMPDPAGPDGHLPAVATLCDLFDREIVVTISWAKSIPGFSSLSLSDQMSVLQSVWMEVLVLGVAQRSLPLQDELAFAEDLVLDEEGARAAGLGELGAALLQLVRRLQALRLEREEYVLLKALALANSDSVHIEDAEAVEQLREALHEALLEYEAGRAGPGGGAERRRAGRLLLTLPLLRQTAGKVLAHFYGVKLEGKVPMHKLFLEMLEAMMD
steroid hormone receptor ERR1 isoform 1
Refseq ID NP_001269379
Protein GI 540344536
UniProt ID P11474
mRNA ID NM_001282450
Length 423
RefSeq Status REVIEWED
Protein sequence is identical to GI:18860920 (mRNA isoform)
steroid hormone receptor ERR1 isoform 2
Refseq ID NP_001269380
Protein GI 540344538
UniProt ID P11474
mRNA ID NM_001282451
Length 422
RefSeq Status REVIEWED
MSSQVVGIEPLYIKAEPASPDSPKGSSETETEPPVALAPGPAPTRCLPGHKEEEDGEGAGPGEQGGGKLVLSSLPKRLCLVCGDVASGYHYGVASCEACKAFFKRTIQGSIEYSCPASNECEITKRRRKACQACRFTKCLRVGMLKEGVRLDRVRGGRQKYKRRPEVDPLPFPGPFPAGPLAVAGGPRKTAPVNALVSHLLVVEPEKLYAMPDPAGPDGHLPAVATLCDLFDREIVVTISWAKSIPGFSSLSLSDQMSVLQSVWMEVLVLGVAQRSLPLQDELAFAEDLVLDEEGARAAGLGELGAALLQLVRRLQALRLEREEYVLLKALALANSDSVHIEDAEAVEQLREALHEALLEYEAGRAGPGGGAERRRAGRLLLTLPLLRQTAGKVLAHFYGVKLEGKVPMHKLFLEMLEAMMD

Gene Information

Entrez Gene ID
2101
Gene Name
estrogen-related receptor alpha
Gene Symbol
ESRRA
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0045171 IDA:HPA C intercellular bridge
GO:0015630 IDA:HPA C microtubule cytoskeleton
GO:0005730 IDA:HPA C nucleolus
GO:0005654 TAS:Reactome C nucleoplasm
GO:0005634 IDA:HPA C nucleus
GO:0003677 TAS:ProtInc F DNA binding
GO:0000978 IEA:Ensembl F RNA polymerase II core promoter proximal region sequence-specific DNA binding
GO:0001078 IEA:Ensembl F RNA polymerase II core promoter proximal region sequence-specific DNA binding transcription factor activity involved in negative regulation of transcription
GO:0001077 IEA:Ensembl F RNA polymerase II core promoter proximal region sequence-specific DNA binding transcription factor activity involved in positive regulation of transcription
GO:0004879 TAS:ProtInc F ligand-activated sequence-specific DNA binding RNA polymerase II transcription factor activity
GO:0019904 IPI:UniProtKB F protein domain specific binding
GO:0043565 IDA:UniProtKB F sequence-specific DNA binding
GO:0005496 IEA:InterPro F steroid binding
GO:0003707 IEA:InterPro F steroid hormone receptor activity
GO:0008270 IEA:InterPro F zinc ion binding
GO:0051216 IEA:Ensembl P cartilage development
GO:0010467 TAS:Reactome P gene expression
GO:0030522 TAS:GOC P intracellular receptor signaling pathway
GO:0045944 ISS:BHF-UCL P positive regulation of transcription from RNA polymerase II promoter
GO:0042127 IEA:Ensembl P regulation of cell proliferation
GO:0045667 IEA:Ensembl P regulation of osteoblast differentiation
GO:0045670 IEA:Ensembl P regulation of osteoclast differentiation
GO:0006355 IMP:UniProtKB P regulation of transcription, DNA-templated
GO:0006367 TAS:Reactome P transcription initiation from RNA polymerase II promoter

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_116145 PPARA activates gene expression
REACT_200608 Transcriptional activation of mitochondrial biogenesis

Domain Information

InterPro Annotations

Accession Description
IPR008946 Nuclear hormone receptor, ligand-binding
IPR000536 Nuclear hormone receptor, ligand-binding, core
IPR024178 Oestrogen receptor/oestrogen-related receptor
IPR027289 Oestrogen-related receptor
IPR001723 Steroid hormone receptor
IPR013088 Zinc finger, NHR/GATA-type
IPR001628 Zinc finger, nuclear hormone receptor-type

UniProt Annotations

Entry Information

Gene Name
estrogen-related receptor alpha
Protein Entry
ERR1_HUMAN
UniProt ID
P11474
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=P11474-1; Sequence=Displayed; Name=2; IsoId=P11474-2; Sequence=VSP_035756; Note=No experimental confirmation available.;
Function Binds 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. {ECO
Induction Induced by PGC1alpha in a number of specific cell types including heart, kidney and muscle.
Interaction Q9Y4A5:TRRAP; NbExp=3; IntAct=EBI-372412, EBI-399128;
Ptm Phosphorylation on Ser-19 enhances sumoylation on Lys-14 increasing repression of transcriptional activity. {ECO
Ptm Reversibly acetylated. Acetylation by PCAF/KAT2 at Lys-129, Lys-138, Lys-160 and Lys-162 and PCAF/KAT2 decreases transcriptional activity probably by inhibiting DNA-binding activity; deacetylation involves SIRT1 and HDAC8 and increases DNA-binding.
Ptm Sumoylated with SUMO2. Main site is Lys-14 which is enhanced by phosphorylation on Ser-19, cofactor activation, and by interaction with PIAS4. Sumoylation enhances repression of transcriptional activity, but has no effect on subcellular location nor on DNA binding. {ECO
Sequence Caution Sequence=AAB17015.1; Type=Erroneous initiation; Evidence= ; Sequence=CAA35778.1; Type=Frameshift; Positions=345, 354; Evidence= ;
Similarity Belongs to the nuclear hormone receptor family. NR3 subfamily.
Similarity Contains 1 nuclear receptor DNA-binding domain.
Subcellular Location Nucleus {ECO
Subunit Binds DNA as a monomer or a homodimer. Interacts (via the AF2 domain) with coactivator PPARGC1A (via the L3 motif); the interaction greatly enhances transriptional activity of genes involved in energy metabolism. Interacts with PIAS4; the interaction enhances sumoylation. {ECO
Web Resource Name=Atlas of Genetics and Cytogenetics in Oncology and Haematology; URL="http://atlasgeneticsoncology.org/Genes/ESRRAID44408ch11q13.html";

Identical and Related Proteins

Unique RefSeq proteins for LMP001124 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
18860920 RefSeq NP_004442 423 steroid hormone receptor ERR1 isoform 1
540344538 RefSeq NP_001269380 422 steroid hormone receptor ERR1 isoform 2

Identical Sequences to LMP001124 proteins

Reference Database Accession Length Protein Name
GI:18860920 DBBJ BAG73124.1 423 estrogen-related receptor alpha, partial [synthetic construct]
GI:18860920 GenBank ABZ92000.1 423 estrogen-related receptor alpha, partial [synthetic construct]
GI:18860920 GenBank ADZ17378.1 423 estrogen-related nuclear receptor alpha [Homo sapiens]
GI:18860920 GenBank AEP61155.1 423 Sequence 149 from patent US 8026065
GI:18860920 RefSeq NP_001269379.1 423 steroid hormone receptor ERR1 isoform 1 [Homo sapiens]
GI:540344538 RefSeq XP_009183996.1 422 PREDICTED: steroid hormone receptor ERR1 [Papio anubis]
GI:540344538 RefSeq XP_003909681.2 422 PREDICTED: steroid hormone receptor ERR1 [Papio anubis]
GI:540344538 RefSeq XP_002821614.3 422 PREDICTED: steroid hormone receptor ERR1 [Pongo abelii]
GI:540344538 RefSeq XP_009244466.1 422 PREDICTED: steroid hormone receptor ERR1 [Pongo abelii]
GI:540344538 RefSeq XP_009421644.1 422 PREDICTED: steroid hormone receptor ERR1 [Pan troglodytes]
GI:540344538 RefSeq XP_010375466.1 422 PREDICTED: steroid hormone receptor ERR1 [Rhinopithecus roxellana]
GI:18860920 SwissProt P11474.3 423 RecName: Full=Steroid hormone receptor ERR1; AltName: Full=Estrogen receptor-like 1; AltName: Full=Estrogen-related receptor alpha; Short=ERR-alpha; AltName: Full=Nuclear receptor subfamily 3 group B member 1 [Homo sapiens]

Related Sequences to LMP001124 proteins

Reference Database Accession Length Protein Name
GI:540344538 GenBank AAB17015.1 481 estrogen receptor-related protein, partial [Homo sapiens]
GI:18860920 GenBank ACM82314.1 438 Sequence 7812 from patent US 6812339
GI:540344538 GenBank AFL62669.1 519 Sequence 1 from patent US 8187871
GI:540344538 GenBank AGN59506.1 519 Sequence 1 from patent US 8455244
GI:18860920 RefSeq XP_001118281.2 423 PREDICTED: steroid hormone receptor ERR1-like isoform 1 [Macaca mulatta]
GI:18860920 RefSeq XP_002799540.1 423 PREDICTED: steroid hormone receptor ERR1-like isoform 2 [Macaca mulatta]
GI:540344538 RefSeq XP_005699916.1 485 PREDICTED: steroid hormone receptor ERR1 isoform X2 [Capra hircus]
GI:18860920 RefSeq XP_006718512.1 492 PREDICTED: steroid hormone receptor ERR1 isoform X1 [Homo sapiens]
GI:540344538 RefSeq XP_006718513.1 491 PREDICTED: steroid hormone receptor ERR1 isoform X2 [Homo sapiens]
GI:18860920 RefSeq XP_006746309.1 423 PREDICTED: steroid hormone receptor ERR1 isoform X1 [Leptonychotes weddellii]
GI:540344538 RefSeq XP_007102665.1 487 PREDICTED: steroid hormone receptor ERR1 [Physeter catodon]
GI:18860920 RefSeq XP_008952224.1 422 PREDICTED: steroid hormone receptor ERR1 [Pan paniscus]