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50 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ESR1
estrogen receptor 1
Chromosome 6 · 6q25.1-q25.2
NCBI Gene: 2099Ensembl: ENSG00000091831.25HGNC: HGNC:3467UniProt: A0A125SXW3
3,639PubMed Papers
21Diseases
51Drugs
5Pathogenic Variants
FUNCTIONAL ROLE
Hub GeneReceptorTranscription Factor
RESEARCH IMPACT
Highly StudiedLandmark GeneTrending
CLINICAL
FDA Approved TargetOMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
estrogen receptor signaling pathwayresponse to estradioltranscription coactivator bindingnegative regulation of canonical NF-kappaB signal transductionbreast cancerosteoporosisbreast carcinomabreast neoplasm
✦AI Summary

ESR1 encodes estrogen receptor alpha (ERα), a nuclear receptor functioning as a ligand-dependent transcription factor in breast tissue. The receptor binds estrogen response elements (EREs) to regulate gene expression and activates endothelial nitric oxide synthase (NOS3) for nitric oxide production 1. ESR1 exists in multiple isoforms that modulate transcriptional activity through competitive binding and heterodimerization mechanisms 2. Clinically, ESR1 mutations represent a critical mechanism of endocrine therapy resistance in hormone receptor-positive metastatic breast cancer (MBC), occurring in up to 36% of patients 3. Common mutations include D538G (36% of cases) and Y537S (14%), which promote ligand-independent, constitutive receptor activation 4. These mutations are selectively enriched during aromatase inhibitor therapy, particularly in the metastatic setting (36.4% prevalence) compared to adjuvant exposure (5.8%) 5. ESR1-mutant tumors demonstrate substantially shorter progression-free survival on subsequent aromatase inhibitor therapy (hazard ratio 3.1) 5. ESR1 mutations can be detected in circulating tumor DNA using digital droplet PCR and next-generation sequencing 6. Recent clinical approvals demonstrate that selective estrogen receptor degraders (SERDs), particularly elacestrant, significantly improve progression-free survival in ESR1-mutant MBC (hazard ratio 0.55) 7, representing a therapeutic breakthrough for this endocrine-resistant phenotype.

Sources cited
1
ESR1 activates NOS3 and endothelial nitric oxide production
PMID: 21937726
2
ESR1 isoforms modulate transcriptional activity through competitive ligand/DNA binding and heterodimerization; bind to ERE
PMID: 10970861
3
ESR1 mutations present in up to 36% of metastatic breast cancer patients; cause constitutive transcriptional activity and reduced endocrine therapy sensitivity
PMID: 34621045
4
D538G is most frequent ESR1 mutation (36%), followed by Y537S (14%); mutations cause constitutive activity and reduced fulvestrant sensitivity
PMID: 27986707
5
ESR1 mutations enriched during metastatic aromatase inhibitor therapy (36.4% vs 5.8% adjuvant); associated with hazard ratio 3.1 for progression-free survival on subsequent AI therapy
PMID: 26560360
6
ESR1 mutations detectable in circulating tumor DNA using digital droplet PCR and next-generation sequencing for liquid biopsy analysis
PMID: 37864956
7
Elacestrant (selective ER degrader) achieves hazard ratio 0.55 for progression-free survival in ESR1-mutant metastatic breast cancer
PMID: 38381994
Disease Associationsⓘ21
breast cancerOpen Targets
0.74Strong
osteoporosisOpen Targets
0.72Strong
breast carcinomaOpen Targets
0.71Strong
breast neoplasmOpen Targets
0.69Moderate
neoplasmOpen Targets
0.67Moderate
endometriosisOpen Targets
0.66Moderate
Estrogen resistance syndromeOpen Targets
0.66Moderate
female infertilityOpen Targets
0.65Moderate
acneOpen Targets
0.61Moderate
postmenopausal osteoporosisOpen Targets
0.61Moderate
menopauseOpen Targets
0.60Moderate
hypogonadismOpen Targets
0.60Moderate
Premature ovarian insufficiencyOpen Targets
0.60Moderate
infertilityOpen Targets
0.60Moderate
primary ovarian insufficiencyOpen Targets
0.59Moderate
AtrophyOpen Targets
0.59Moderate
contraceptionOpen Targets
0.59Moderate
DyspareuniaOpen Targets
0.58Moderate
polycystic ovary syndromeOpen Targets
0.57Moderate
postmenopausal atrophic vaginitisOpen Targets
0.55Moderate
Estrogen resistanceUniProt
Pathogenic Variants5
NM_000125.4(ESR1):c.453-351A>GPathogenic
Estrogen resistance syndrome|Scoliosis, isolated, susceptibility to, 1
★☆☆☆
NM_000125.4(ESR1):c.1181G>A (p.Arg394His)Pathogenic
Estrogen resistance syndrome
☆☆☆☆2018→ Residue 394
NM_000125.4(ESR1):c.1125G>T (p.Gln375His)Pathogenic
Estrogen resistance syndrome
☆☆☆☆2013→ Residue 375
NM_000125.4(ESR1):c.469C>T (p.Arg157Ter)Pathogenic
Estrogen resistance syndrome
☆☆☆☆1994→ Residue 157
NM_000125.4(ESR1):c.1339_1340delinsGC (p.Cys447Ala)Pathogenic
Estrogen receptor mutant, temperature-sensitive
☆☆☆☆1992→ Residue 447
View on ClinVar ↗
Drug Targets51
ACOLBIFENEPhase III
Estrogen receptor modulator
breast cancer
AFIMOXIFENEPhase II
Estrogen-related receptor gamma antagonist
breast cancer
AMCENESTRANTPhase III
Estrogen receptor alpha degrader
breast cancer
ARZOXIFENEApproved
Estrogen receptor modulator
breast cancer
BAZEDOXIFENEApproved
Estrogen receptor modulator
osteopetrosis
BAZEDOXIFENE ACETATEApproved
Estrogen receptor modulator
osteoporosis
BRILANESTRANTPhase II
Estrogen receptor degrader
breast cancer
CAMIZESTRANTPhase III
Estrogen receptor alpha degrader
breast cancer
CHF4227Phase I
Estrogen receptor modulator
osteoporosis
CLOMIPHENEApproved
Estrogen receptor alpha modulator
female infertility
CLOMIPHENE CITRATEApproved
Estrogen receptor alpha modulator
infertility
CYCLOFENILApproved
Estrogen receptor modulator
infertility
DIENESTROLApproved
Estrogen receptor alpha agonist
Atrophic Vaginitis
DIETHYLSTILBESTROLApproved
Estrogen receptor alpha agonist
DIETHYLSTILBESTROL DIPHOSPHATEApproved
Estrogen receptor agonist
DROLOXIFENEPhase II
Estrogen receptor modulator
breast cancer
ELACESTRANTApproved
Estrogen receptor alpha degrader
breast cancer
ELACESTRANT HYDROCHLORIDEApproved
Estrogen receptor alpha degrader
male breast carcinoma
ENCLOMIPHENEPhase II
Estrogen receptor alpha modulator
obesity
ESTETROLApproved
Estrogen receptor agonist
ESTETROL ANHYDROUSUNKNOWN
Estrogen receptor agonist
contraception
ESTRADIOLApproved
Estrogen receptor alpha agonist
Atrophy
ESTRADIOL ACETATEApproved
Estrogen receptor alpha agonist
Atrophy
ESTRADIOL CYPIONATEApproved
Estrogen receptor alpha agonist
ESTRADIOL VALERATEApproved
Estrogen receptor alpha agonist
Menorrhagia
ESTRIOLApproved
Estrogen receptor modulator
ESTROGENS, CONJUGATEDApproved
Estrogen receptor agonist
Atrophy
ESTROGENS, CONJUGATED SYNTHETIC AApproved
Estrogen receptor agonist
Atrophy
ESTROGENS, ESTERIFIEDApproved
Estrogen receptor agonist
breast cancer
ESTRONEApproved
Estrogen receptor alpha agonist
ESTROPIPATEApproved
Estrogen receptor alpha agonist
Atrophy
ETHINYL ESTRADIOLApproved
Estrogen receptor alpha agonist
acne
FISPEMIFENEPhase II
Estrogen receptor modulator
hypogonadism
FULVESTRANTApproved
Estrogen receptor alpha antagonist
breast cancer
GIREDESTRANTPhase III
Estrogen receptor alpha degrader
breast cancer
GTX-758Phase II
Estrogen receptor alpha agonist
prostate cancer
IDOXIFENEPhase III
Estrogen receptor modulator
breast cancer
LASOFOXIFENEApproved
Estrogen receptor modulator
osteoporosis
LASOFOXIFENE TARTRATEApproved
Estrogen receptor modulator
postmenopausal osteoporosis
MESTRANOLApproved
Estrogen receptor alpha agonist
contraception
MK-6913Phase II
Estrogen receptor agonist
OSPEMIFENEApproved
Estrogen receptor modulator
POLYESTRADIOL PHOSPHATEApproved
Estrogen receptor alpha agonist
prostate cancer
QUINESTROLApproved
Estrogen receptor agonist
breast cancer
RALOXIFENEApproved
Estrogen receptor beta modulator
osteoporosis
SYNTHETIC CONJUGATED ESTROGENS, BApproved
Estrogen receptor agonist
Atrophy
TAMOXIFENApproved
Estrogen receptor alpha modulator
breast cancer
TAMOXIFEN CITRATEApproved
Estrogen receptor alpha modulator
TAS-108Phase II
Estrogen receptor alpha antagonist
breast cancer
TOREMIFENEApproved
Estrogen receptor modulator
breast cancer
TOREMIFENE CITRATEApproved
Estrogen receptor modulator
breast cancer
Related Genes
SFNProtein interaction100%NR2F1Protein interaction100%FKBPLProtein interaction100%TP53Protein interaction100%JUNProtein interaction100%TLE3Protein interaction100%
Tissue Expression6 tissues
Liver
100%
Ovary
76%
Heart
16%
Lung
15%
Bone Marrow
11%
Brain
2%
Gene Interaction Network
Click a node to explore
ESR1SFNNR2F1FKBPLTP53JUNTLE3
PROTEIN STRUCTURE
Preparing viewer…
PDB7BAA · 1.10 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.46Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.30 [0.20–0.46]
RankingsWhere ESR1 stands among ~20K protein-coding genes
  • #12of 20,598
    Most Researched3,639 · top 1%
  • #47of 1,025
    FDA-Approved Drug Targets36 · top 5%
  • #3,452of 5,498
    Most Pathogenic Variants5
  • #2,557of 17,882
    Most Constrained (LOEUF)0.46 · top quartile
Genes detectedESR1
Sources retrieved50 papers
Response time—
📄 Sources
50▼
1
ESR1 mutations and therapeutic resistance in metastatic breast cancer: progress and remaining challenges.
PMID: 34621045
Br J Cancer · 2022
1.00
2
PMID: 32546646
Clin Cancer Res · 2020
0.90
3
ESR1 mutations in breast cancer.
PMID: 31318440
Cancer · 2019
0.90
4
Enhanced prostatic Esr1
PMID: 38606616
J Pathol · 2024
0.88
5
ESR1 testing on FFPE samples from metastatic lesions in HR + /HER2- breast cancer after progression on CDK4/6 inhibitor therapy.
PMID: 40369610
Breast Cancer Res · 2025
0.86