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26 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ESRRA
estrogen related receptor alpha
Chromosome 11 · 11q13.1
NCBI Gene: 2101Ensembl: ENSG00000173153.18HGNC: HGNC:3471UniProt: P11474
228PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
ReceptorTranscription Factor
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
regulation of DNA-templated transcriptionfibrillar centernucleoplasmcytoplasmadenoid cystic carcinomabreast cancerneoplasmtriple-negative breast cancer
✦AI Summary

ESRRA (estrogen related receptor alpha) is an orphan nuclear receptor that functions as a master regulator of cellular metabolism and energy homeostasis through transcriptional control mechanisms 1. The protein operates through dual mechanisms: binding to estrogen response element-related sequences (ERREs) to activate transcription of metabolic genes, and forming regulatory complexes with coactivators like PGC1A 2. ESRRA plays critical roles in mitochondrial biogenesis and oxidative phosphorylation by transcriptionally activating genes involved in fatty acid oxidation and OXPHOS pathways 3. The receptor also regulates autophagy and mitophagy through transcriptional activation of ATG5, which is essential for maintaining cellular homeostasis under metabolic stress 4. Additionally, ESRRA controls ribosomal protein expression, specifically RPLP1, to mediate adaptive protein translation during prolonged starvation 5. In disease contexts, ESRRA dysfunction contributes to multiple pathological conditions including diabetic kidney disease, inflammatory bowel disease, and cancer progression 67. The receptor's expression is dynamically regulated during development and disease states, with therapeutic targeting showing promise in metabolic disorders and cancer treatment. ESRRA's multifaceted roles in energy metabolism, autophagy regulation, and cellular adaptation make it a crucial mediator of metabolic homeostasis.

Sources cited
1
ESRRA is a key regulator responding to metabolic stress and affects bone marrow adipocyte function
PMID: 38704393
2
ESRRA forms regulatory complexes and controls mitochondrial oxidation genes in cardiac function
PMID: 39747914
3
ESRRA is critical in mitochondrial biogenesis and autophagy function, regulating intestinal homeostasis
PMID: 33172329
4
ESRRA transcriptionally activates ATG5 to sustain mitophagy and preserve tubular homeostasis
PMID: 41376268
5
ESRRA serves as master regulator of adaptive translation by transcriptionally stimulating RPLP1
PMID: 39936615
6
ESRRA orchestrates transcriptional activation of ERα-occupied super enhancers and regulates interferon signaling
PMID: 41111053
7
KDM5 inhibition increases ESRRA expression and enhances genes involved in fatty acid oxidation and OXPHOS
PMID: 38230606
8
ESRRA binding sites are enriched in NAFLD-associated methylation changes after bariatric surgery
PMID: 23931760
Disease Associationsⓘ20
adenoid cystic carcinomaOpen Targets
0.22Weak
breast cancerOpen Targets
0.12Weak
neoplasmOpen Targets
0.11Weak
triple-negative breast cancerOpen Targets
0.11Weak
prostate cancerOpen Targets
0.11Weak
posterior cortical atrophyOpen Targets
0.10Suggestive
osteosarcomaOpen Targets
0.10Suggestive
endometrial cancerOpen Targets
0.09Suggestive
ovarian cancerOpen Targets
0.09Suggestive
gastric cancerOpen Targets
0.08Suggestive
cancerOpen Targets
0.08Suggestive
non-alcoholic fatty liver diseaseOpen Targets
0.08Suggestive
oral squamous cell carcinomaOpen Targets
0.08Suggestive
non-small cell lung carcinomaOpen Targets
0.08Suggestive
diabetic nephropathyOpen Targets
0.08Suggestive
lung adenocarcinomaOpen Targets
0.08Suggestive
myocarditisOpen Targets
0.08Suggestive
viral diseaseOpen Targets
0.07Suggestive
renal cell carcinomaOpen Targets
0.07Suggestive
non-alcoholic steatohepatitisOpen Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PPARGC1AProtein interaction100%PPARGProtein interaction100%ALAS1Protein interaction100%NCOR1Protein interaction100%NRIP1Protein interaction99%ESRRGProtein interaction97%
Tissue Expression6 tissues
Liver
100%
Heart
69%
Lung
53%
Brain
16%
Bone Marrow
11%
Ovary
9%
Gene Interaction Network
Click a node to explore
ESRRAPPARGC1APPARGALAS1NCOR1NRIP1ESRRG
PROTEIN STRUCTURE
Preparing viewer…
PDB3K6P · 2.00 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.46Moderately Constrained
pLIⓘ
0.99Intolerant
Observed/Expected LoF0.24 [0.14–0.46]
RankingsWhere ESRRA stands among ~20K protein-coding genes
  • #1,769of 20,598
    Most Researched228 · top 10%
  • #2,575of 17,882
    Most Constrained (LOEUF)0.46 · top quartile
Genes detectedESRRA
Sources retrieved26 papers
Response time—
📄 Sources
26▼
1
Targeting adipocyte ESRRA promotes osteogenesis and vascular formation in adipocyte-rich bone marrow.
PMID: 38704393
Nat Commun · 2024
1.00
2
The transcriptional repressor HEY2 regulates mitochondrial oxidative respiration to maintain cardiac homeostasis.
PMID: 39747914
Nat Commun · 2025
0.90
3
The pro-inflammatory cytokine IL6 suppresses mitochondrial function via the gp130-JAK1/STAT1/3-HIF1α/ERRα axis.
PMID: 40056415
Cell Rep · 2025
0.80
4
ESRRA (estrogen related receptor alpha) is a critical regulator of intestinal homeostasis through activation of autophagic flux via gut microbiota.
PMID: 33172329
Autophagy · 2021
0.80
5
ESRRA-ATG5-Mediated mitophagy enhances arginine metabolism to alleviate diabetic kidney disease.
PMID: 41376268
Autophagy · 2026
0.70