Estrogen Related Receptor Alpha (ERRa)

ERR1; ERRa; ERRalpha; ESRL1; NR3B1; Estrogen receptor-like 1; Nuclear receptor subfamily 3 group B member 1

SPN	S829
HGNC	3471
MGI	1346831
RGD	1583866
UniProt	P11474

ERRα has wide tissue distribution but it is most highly expressed in tissue that preferentially use fatty acids as energy sources such as kidney, heart, cerebellum, intestine, and skeletal muscle. 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 transcription 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.

Reference

"Identification of a new class of steroid hormone receptors."
Nature 331:91-94(1988) [PubMed] [Europe PMC] [Abstract]

"Estrogen-related receptor, hERR1, modulates estrogen receptor-mediated response of human lactoferrin gene promoter."
J. Biol. Chem. 271:5795-5804(1996) [PubMed] [Europe PMC] [Abstract]

"Human chromosome 11 DNA sequence and analysis including novel gene identification."
Nature 440:497-500(2006) [PubMed] [Europe PMC] [Abstract]

"SV40 early-to-late switch involves titration of cellular transcriptional repressors."
Genes Dev. 7:2206-2219(1993) [PubMed] [Europe PMC] [Abstract]

"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. 17:5400-5409(1997) [PubMed] [Europe PMC] [Abstract]

"The transcriptional coactivator PGC-1 regulates the expression and activity of the orphan nuclear receptor estrogen-related receptor alpha (ERRalpha)."
J. Biol. Chem. 278:9013-9018(2003) [PubMed] [Europe PMC] [Abstract]

"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. 20:302-310(2006) [PubMed] [Europe PMC] [Abstract]

"Phosphorylation-dependent sumoylation of estrogen-related receptor alpha1."
Biochemistry 46:9795-9804(2007) [PubMed] [Europe PMC] [Abstract]

"Phosphorylation-dependent sumoylation regulates estrogen-related receptor-alpha and -gamma transcriptional activity through a synergy control motif."
Mol. Endocrinol. 22:570-584(2008) [PubMed] [Europe PMC] [Abstract]

"A quantitative atlas of mitotic phosphorylation."
Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]

"An acetylation switch modulates the transcriptional activity of estrogen-related receptor alpha."
Mol. Endocrinol. 24:1349-1358(2010) [PubMed] [Europe PMC] [Abstract]

"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. 288:25207-25218(2013) [PubMed] [Europe PMC] [Abstract]

"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. 279:49330-49337(2004) [PubMed] [Europe PMC] [Abstract]

"Communication between the ERRalpha homodimer interface and the PGC-1alpha binding surface via the helix 8-9 loop."
J. Biol. Chem. 283:20220-20230(2008) [PubMed] [Europe PMC] [Abstract]