ESR1 Gene Complete Identifier and Functional Mapping Reference

Provide a comprehensive cross-database identifier and functional mapping reference for human ESR1 — a definitive lookup resource covering: ### …

Provide a comprehensive cross-database identifier and functional mapping reference for human ESR1 — a definitive lookup resource covering: ### Section 1: Gene identifiers For human gene ESR1, list ALL gene-level database identifiers. Required: - HGNC ID and approved symbol - Ensembl gene ID (ENSG...) - NCBI Entrez Gene ID - OMIM gene/locus ID - Genomic location: chromosome, start position, end position, strand (GRCh38) ### Section 2: Transcript identifiers For human gene ESR1, list ALL transcript-level identifiers. Required: - Ensembl transcripts: ALL ENST IDs with biotype. Total count. - RefSeq transcripts: ALL NM_ mRNA accessions. Mark which is MANE Select. - CCDS IDs. - For the CANONICAL/MANE SELECT transcript: ALL exon IDs (ENSE) with genomic coordinates and total exon count. ### Section 3: Protein identifiers For human gene ESR1 protein product(s), list ALL protein-level identifiers. Required: - UniProt accessions: ALL entries (reviewed and unreviewed). Mark the canonical reviewed entry. - RefSeq protein: ALL NP_ accessions. - Protein domains and families: list ALL annotated domains/families with identifiers, including name, type (domain/family/superfamily), and ID. - Antibody availability: known antibody resources for the protein. ### Section 4: Structure For human gene ESR1 protein, list ALL structural data. Required: - Experimental structures: ALL PDB IDs. For each: experimental method (X-ray/NMR/Cryo-EM) and resolution. Total count. - Predicted structures: AlphaFold model ID and confidence metrics (pLDDT). ### Section 5: Cross-species orthologs For human gene ESR1, list orthologous genes in key model organisms. Organisms: - Mouse (Mus musculus): gene ID, symbol - Rat (Rattus norvegicus): gene ID, symbol - Zebrafish (Danio rerio): gene ID, symbol - Fruit fly (Drosophila melanogaster): gene ID, symbol - Worm (C. elegans): gene ID, symbol - Yeast (S. cerevisiae): gene ID, symbol ### Section 6: Clinical variants & AI predictions For human gene ESR1, summarize clinical variants and AI predictions. Clinical variant annotations (ClinVar): - Total variant count (approximate is fine) - Breakdown by classification: Pathogenic, Likely Pathogenic, VUS, Likely Benign, Benign - TOP 30 pathogenic/likely pathogenic variants with: variant ID, HGVS notation, associated condition AI-based variant effect predictions: - Splice effect predictions: total count + TOP 30 with delta scores if known - Missense pathogenicity from AlphaMissense — total count + TOP 30 likely-pathogenic with am_pathogenicity scores. ### Section 7: Pathways & Gene Ontology For human gene ESR1, list biological pathways and Gene Ontology annotations. Pathway membership: - ALL biological pathways this gene participates in, with pathway IDs and names - Total pathway count Gene Ontology: - Biological Process: count and TOP 20 terms with GO IDs - Molecular Function: count and TOP 20 terms with GO IDs - Cellular Component: count and TOP 20 terms with GO IDs ### Section 8: Protein interactions & networks For human gene ESR1 protein, summarize protein interactions and networks. Protein-protein interactions (STRING, IntAct, BioGRID, etc.): - Total interaction count (approximate) - TOP 30 highest-confidence interacting proteins with scores/evidence Protein similarity: - Structural/embedding similarity (e.g. Foldseek, ESM): TOP 20 similar proteins with scores - Sequence homology: TOP 20 homologous proteins with identity/similarity ### Section 9: Transcription factor regulatory data For human gene ESR1, summarize transcription factor regulatory data. If ESR1 is a transcription factor: - Downstream targets: total count + TOP 30 with regulation type (activates/represses) and evidence - DNA binding motifs from JASPAR — all known motif IDs and motif family classification. Regardless: - Upstream regulators: TFs that regulate ESR1 — names with evidence type (ChIP-seq / predicted / experimentally validated) If ESR1 is not a transcription factor, say so briefly and skip the downstream/motif sections. ### Section 10: Drug & pharmacology data For human gene ESR1 protein as a drug target, summarize pharmacology data. If ESR1 is a known drug target: - Targeting molecules: total count in ChEMBL/DrugBank + TOP 30 by development phase (molecule ID, name, mechanism, highest phase) - Clinical trials: TOP 20 involving drugs targeting this gene — trial ID, phase, status, intervention - Pharmacogenomics: known drug-gene interactions affecting drug response + dosing guidelines if any If ESR1 is not currently a drug target, say so briefly. ### Section 11: Expression profiles For human gene ESR1, summarize expression profiles. Tissue expression (GTEx, HPA, Bgee, etc.): - TOP 30 tissues with expression scores/levels (direction, units if known) - Note tissue-specific or tissue-enriched patterns Cell type expression (Tabula Sapiens, HCA, etc.): - TOP 30 cell types with expression scores - Note cell-type-specific patterns Single-cell expression: notable datasets or cell populations of interest for this gene. ### Section 12: Disease associations For human gene ESR1, summarize disease associations. Mendelian / monogenic disease: - Diseases caused by mutations in ESR1: disease name, disease ID (OMIM/Orphanet/Mondo), inheritance pattern, evidence level - Include all directly linked conditions Phenotype associations: - Clinical phenotypes associated with the gene (HPO terms where known) - TOP 30 phenotype terms with HPO IDs Complex-disease / GWAS: - Traits and diseases significantly associated via GWAS: trait name, variant, effect size, study where known - TOP 30 GWAS associations

ESR1

Executive summary

ESR1 encodes estrogen receptor alpha (ER-alpha, UniProt P03372), the primary mediator of estrogen signaling in humans and one of the most clinically important targets in oncology. Located on chromosome 6 (151,651,284–152,129,619 bp), it functions as a ligand-activated transcription factor with zinc finger DNA-binding and nuclear hormone receptor ligand-binding domains, directly activating or repressing at least 100 downstream target genes. Expression is highest in female reproductive tissues (oviduct, cervix, endometrium, breast epithelium) but extends broadly across 286 conditions. ESR1 is the target of major approved breast cancer therapeutics including tamoxifen (241 clinical trials), fulvestrant (418 trials), and letrozole (555 trials), with CYP2D6 pharmacogenomics critically affecting tamoxifen efficacy. GWAS links ESR1 variants to heel bone mineral density (p=2e-212), breast cancer susceptibility (p=5e-54), and cardiovascular traits, while rare loss-of-function mutations cause autosomal recessive estrogen resistance syndrome. The protein is exceptionally well-studied structurally, with 475 experimental PDB entries and an 8,546-partner STRING interaction network anchored by coactivators, BRCA1, SRC, and EGFR.

ESR1 — Reference

Cross-database identifier and functional mapping reference for ESR1.

Gene identifiers

  • HGNC ID: HGNC:3467
  • Approved symbol: ESR1
  • Ensembl gene ID: ENSG00000091831
  • NCBI Entrez Gene ID: 2099
  • OMIM gene/locus ID: 133430
  • Genomic location (GRCh38):
    • Chromosome: 6
    • Start position: 151,651,284 bp
    • End position: 152,129,619 bp
    • Strand: +

Transcript identifiers

Ensembl transcripts (18 total)

Transcript IDBiotype
ENST00000206249protein_coding
ENST00000338799protein_coding
ENST00000404742protein_coding
ENST00000406599protein_coding
ENST00000415488protein_coding
ENST00000427531protein_coding
ENST00000440973protein_coding
ENST00000443427protein_coding
ENST00000446550protein_coding
ENST00000456483protein_coding
ENST00000473497protein_coding_CDS_not_defined
ENST00000482101protein_coding_CDS_not_defined
ENST00000488573protein_coding_CDS_not_defined
ENST00000641399protein_coding_CDS_not_defined
ENST00000858333protein_coding
ENST00000858334protein_coding
ENST00000858335protein_coding
ENST00000947759protein_coding

RefSeq mRNA transcripts (NM_ accessions)

AccessionMANE Select
NM_000125✓ Yes
NM_001122740No
NM_001122741No
NM_001122742No
NM_001291230No
NM_001291241No
NM_001328100No
NM_001385568No
NM_001385569No
NM_001385570No
NM_001385571No
NM_001385572No

CCDS identifiers

CCDS ID
CCDS5234
CCDS87457

MANE Select/Canonical transcript exons: ENST00000206249 (NM_000125)

Total exon count: 8

Exon IDStartEndStrandChromosome
ENSE00001877305151,807,682151,808,364+6
ENSE00003705805151,842,597151,842,787+6
ENSE00003721187151,880,655151,880,771+6
ENSE00003743537152,060,991152,061,124+6
ENSE00003736522152,094,385152,094,568+6
ENSE00000813753152,011,656152,011,794+6
ENSE00001128501152,098,732152,103,274+6

Protein identifiers

UniProt accessions

Canonical reviewed entry:

  • P03372 — Estrogen receptor (ER-alpha)

Unreviewed entries:

  • B0QYW7
  • G4XH65
  • H0Y4W6
  • Q5T5H8
  • Q9H2M1
  • Q9H2M2
  • Q9UE35

RefSeq protein accessions (NP_)

  • NP_000116 — MANE Select canonical
  • NP_001116212
  • NP_001116213
  • NP_001116214
  • NP_001278159
  • NP_001278170
  • NP_001315029
  • NP_001372497
  • NP_001372498

Protein domains and families

IdentifierTypeDatabaseName
IPR000536DomainInterProNuclear hormone receptor, ligand-binding domain
IPR001292FamilyInterProEstrogen receptor
IPR001628DomainInterProZinc finger, nuclear hormone receptor-type
IPR001723FamilyInterProNuclear hormone receptor
IPR013088Homologous superfamilyInterProZinc finger, NHR/GATA-type
IPR024178FamilyInterProEstrogen receptor/oestrogen-related receptor
IPR024736DomainInterProOestrogen-type nuclear receptor final C-terminal domain
IPR035500Homologous superfamilyInterProNuclear hormone receptor-like domain superfamily
IPR046944DomainInterProEstrogen receptor, N-terminal
IPR050200FamilyInterProNuclear hormone receptor family NR3 subfamily
PF00104DomainPfam
PF00105DomainPfam
PF02159DomainPfam
PF12743DomainPfam
SM00399DomainSMART(Zinc finger, nuclear hormone receptor-type)
SM00430DomainSMART(Nuclear hormone receptor, ligand-binding domain)
CD06949DomainCDD
CD07171DomainCDD

Antibody availability

No antibody resources found in biobtree for ESR1. The standard biobtree antibody database does not contain entries mapping to this protein.

Structure

Experimental Structures: 475 total PDB entries

X-ray Crystallography

  • Total count: 468 structures
  • Resolution range: 1.15 – 3.1 Å
  • Notable high-resolution structures:
    • Highest resolution: 7B9R, 7B9T, 7BA8, 7BA9, 8BZC, 8BZW, 8C04 (1.15 Å)
    • Typical range: 1.4 – 2.5 Å
  • Ligand complexes: Predominantly ligand-binding domain (LBD) structures with various estrogens, selective estrogen receptor modulators (SERMs), selective estrogen receptor degraders (SERDs), and synthetic compounds
  • Key regions covered: Ligand-binding domain, DNA-binding domain interactions with proteins and peptides

Solution NMR

  • Total count: 4 structures
  • Entries: 1HCP, 2LLO, 2LLQ, 5T0X
  • Note: No X-ray resolution data; these represent solution structures of DNA-binding domain and calmodulin-binding peptide complexes

Predicted Structure: AlphaFold

  • Model ID: P03372
  • Confidence metric (pLDDT): 67.14 (global average)
  • High confidence regions: 45% of the structure (pLDDT > 90)
  • Interpretation: Moderate overall confidence with well-predicted structured domains; lower confidence in flexible regions typical of intrinsically disordered proteins

Cross-species orthologs

OrganismGene IDSymbol
Mouse (Mus musculus)13982Esr1
Rat (Rattus norvegicus)24890Esr1
Zebrafish (Danio rerio)259252esr1
Fruit fly (Drosophila melanogaster)38912ERR
Worm (C. elegans)nonenone
Yeast (S. cerevisiae)nonenone

Clinical variants & AI predictions

ClinVar Summary

Total variants: ~217

Classification breakdown:

ClassCount
Benign33
Likely Benign32
Uncertain Significance (VUS)~140
Pathogenic2
Likely Pathogenic0
Risk Factor2
Conflicting2

Top 30 Pathogenic/Likely Pathogenic variants (ranked by classification strength):

ClinVar IDHGVS NotationCondition/Note
16590NM_000125.4(ESR1):c.1339_1340delinsGC (p.Cys447Ala)Pathogenic
16592NM_000125.4(ESR1):c.469C>T (p.Arg157Ter)Pathogenic

Note: Only 2 pathogenic/likely pathogenic variants in current ClinVar database for ESR1. Remaining disease associations documented primarily as VUS (n=~140).


AlphaMissense Predictions

Total variants: 3,910

Likely pathogenic variants: 117+ (filtered subset shown)

Top 30 likely-pathogenic missense predictions:

VariantProtein ChangeAlphaMissense Score
6:151842697:T:CC185R1.000
6:151842697:T:AC185S1.000
6:151842697:T:GC185G1.000
6:151842698:G:AC185Y1.000
6:151842698:G:CC185S1.000
6:151842698:G:TC185F1.000
6:151842699:T:GC185W1.000
6:151842706:T:AC188S1.000
6:151842706:T:CC188R1.000
6:151842707:G:AC188Y1.000
6:151842707:G:CC188S1.000
6:151842707:G:TC188F1.000
6:151842708:C:GC188W1.000
6:151842712:G:CD190H1.000
6:151842713:A:CD190A1.000
6:151842713:A:GD190G1.000
6:151842713:A:TD190V1.000
6:151842715:T:CY191H0.936
6:151842685:G:AE181K0.952
6:151842684:G:CK180N0.925
6:151842684:G:TK180N0.925
6:151842686:A:TE181V0.922
6:151842691:C:AR183S0.984
6:151842692:G:CR183P0.982
6:151842700:G:CA186P0.996
6:151842701:C:AA186E0.964
6:151842703:G:AV187M0.999
6:151842703:G:CV187L0.999
6:151842703:G:TV187L0.999
6:151842704:T:AV187E1.000

Splice Effect Predictions (SpliceAI)

Total predicted splice variants: 4,481

Top 30 high-confidence splice predictions (Delta score ≥ 0.8):

Genomic PositionEffect TypeDelta Score
6:151690665:G:GGdonor_gain1.00
6:151690595:TCC:Tdonor_gain0.92
6:151690663:AAG:Adonor_loss0.98
6:151690664:AG:Adonor_loss0.98
6:151690665:G:Adonor_loss0.98
6:151690666:TAG:Tdonor_loss0.98
6:151690669:G:GTdonor_loss0.81
6:151690596:C:Adonor_gain0.79
6:151690649:A:AGdonor_gain0.77
6:151690660:TTCAA:Tdonor_gain0.84
6:151699907:A:Gdonor_gain0.83
6:151698861:G:GTdonor_gain0.45
6:151701208:G:GTdonor_gain0.32
6:151690663:AA:Adonor_gain0.90
6:151690662:CAA:Cdonor_gain0.67
6:151690662:CAAGT:Cdonor_gain0.62
6:151697494:G:GTdonor_gain0.62
6:151690666:T:Cdonor_gain0.62
6:151697479:A:AGdonor_gain0.63
6:151690668:GGTA:Gdonor_loss0.94
6:151692211:ATTG:Aacceptor_gain0.52
6:151695288:CTA:Cacceptor_gain0.42
6:151697288:TAAAG:Tacceptor_gain0.41
6:151697289:AAAGA:Aacceptor_gain0.41
6:151698675:C:Tacceptor_gain0.46
6:151690791:T:Aacceptor_gain0.41
6:151690798:A:G(no score)
6:151690821:C:Tdonor_gain0.53
6:151690863:AC:Adonor_gain0.66
6:151692211:ATT:Aacceptor_gain0.47

Pathways & Gene Ontology

Reactome Pathways

ESR1 participates in 17 Reactome pathways:

IDPathway Name
R-HSA-1251985Nuclear signaling by ERBB4
R-HSA-1257604PIP3 activates AKT signaling
R-HSA-2219530Constitutive Signaling by Aberrant PI3K in Cancer
R-HSA-383280Nuclear Receptor transcription pathway
R-HSA-4090294SUMOylation of intracellular receptors
R-HSA-5689896Ovarian tumor domain proteases
R-HSA-6811558PI5P, PP2A and IER3 Regulate PI3K/AKT Signaling
R-HSA-8866910TFAP2 (AP-2) family regulates transcription of growth factors and their receptors
R-HSA-8931987RUNX1 regulates estrogen receptor mediated transcription
R-HSA-8939211ESR-mediated signaling
R-HSA-8939256RUNX1 regulates transcription of genes involved in WNT signaling
R-HSA-8939902Regulation of RUNX2 expression and activity
R-HSA-9009391Extra-nuclear estrogen signaling
R-HSA-9018519Estrogen-dependent gene expression
R-HSA-9841251Mitochondrial unfolded protein response (UPRmt)
R-HSA-9927418Developmental Lineage of Mammary Gland Luminal Epithelial Cells
R-HSA-9927426Developmental Lineage of Mammary Gland Alveolar Cells

MSigDB Gene Sets

ESR1 is a member of 300+ MSigDB gene sets including:

  • GO-based sets (Biological Process, Molecular Function, Cellular Component)
  • Curated pathway sets (including PID, BIOCARTA, REACTOME subsets)
  • Transcription factor targets
  • miRNA targets
  • Cancer-related signatures (breast cancer luminal, endocrine therapy resistance, ESR1-specific sets)
  • Immunological signatures
  • Developmental and reproductive process sets

Gene Ontology Annotations

Total: 80 GO terms annotated for ESR1 via UniProt

Biological Process (43 terms)

RankGO IDTerm
1GO:0030520Estrogen receptor signaling pathway
2GO:0045944Positive regulation of transcription by RNA polymerase II
3GO:0006357Regulation of transcription by RNA polymerase II
4GO:0043401Steroid hormone receptor signaling pathway
5GO:0043627Response to estrogen
6GO:0071391Cellular response to estrogen stimulus
7GO:0071392Cellular response to estradiol stimulus
8GO:0032355Response to estradiol
9GO:0007165Signal transduction
10GO:0045893Positive regulation of DNA-templated transcription
11GO:0030518Nuclear receptor-mediated steroid hormone signaling pathway
12GO:0007200Phospholipase C-activating G protein-coupled receptor signaling pathway
13GO:0051123RNA polymerase II preinitiation complex assembly
14GO:0048863Stem cell differentiation
15GO:0048146Positive regulation of fibroblast proliferation
16GO:0006338Chromatin remodeling
17GO:0000122Negative regulation of transcription by RNA polymerase II
18GO:0045429Positive regulation of nitric oxide biosynthetic process
19GO:0060749Mammary gland alveolus development
20GO:0060750Epithelial cell proliferation involved in mammary gland duct elongation

Molecular Function (25 terms)

RankGO IDTerm
1GO:0004879Nuclear receptor activity
2GO:0030284Nuclear estrogen receptor activity
3GO:0000981DNA-binding transcription factor activity, RNA polymerase II-specific
4GO:0001228DNA-binding transcription activator activity, RNA polymerase II-specific
5GO:0003700DNA-binding transcription factor activity
6GO:0034056Estrogen response element binding
7GO:0030331Nuclear estrogen receptor binding
8GO:0001221Transcription coregulator binding
9GO:0001223Transcription coactivator binding
10GO:0001222Transcription corepressor binding
11GO:0005496Steroid binding
12GO:0000978RNA polymerase II cis-regulatory region sequence-specific DNA binding
13GO:0003682Chromatin binding
14GO:0008013Beta-catenin binding
15GO:0008270Zinc ion binding
16GO:0019899Enzyme binding
17GO:0019901Protein kinase binding
18GO:0042802Identical protein binding
19GO:0005516Calmodulin binding
20GO:0017025TBP-class protein binding

Cellular Component (11 terms)

RankGO IDTerm
1GO:0005634Nucleus
2GO:0005654Nucleoplasm
3GO:0005737Cytoplasm
4GO:0005829Cytosol
5GO:0000785Chromatin
6GO:0000791Euchromatin
7GO:0005667Transcription regulator complex
8GO:0016020Membrane
9GO:0005886Plasma membrane
10GO:0005794Golgi apparatus
11GO:0032991Protein-containing complex

Protein interactions & networks

Protein-Protein Interactions

Total Interaction Count:

  • STRING: 8,546 interactions
  • BioGRID: ~1,384 interactions
  • IntAct: 392 interactions

TOP 30 STRING Interactions (Highest Confidence Scores)

RankGene SymbolUniProt IDSTRING ScoreProtein Name
1NCOA3Q9Y6Q9997Nuclear receptor coactivator 3
2SRCP12931996Proto-oncogene tyrosine-protein kinase Src
3JUNP05412995Transcription factor Jun
4BRCA1P38398995Breast cancer type 1 susceptibility protein
5FOSP01100994Protein c-Fos
6IGF1RP08069994Insulin-like growth factor 1 receptor
7CAV1Q03135992Caveolin-1
8FOXA1P55317990Hepatocyte nuclear factor 3-alpha
9HSP90AA1P07900988Heat shock protein HSP 90-alpha
10NRIP1P48552988Nuclear receptor-interacting protein 1
11NCOR1O75376987Nuclear receptor corepressor 1
12HDAC1Q13547987Histone deacetylase 1
13NCOA1Q15788986Nuclear receptor coactivator 1
14GNA13Q14344985Guanine nucleotide-binding protein subunit alpha-13
15HSP90AB1P08238983Heat shock protein HSP 90-beta
16CALM3P27482981Calmodulin-like protein 3
17SP1P08047979Transcription factor Sp1
18ARP10275979Androgen receptor
19NCOA2Q15596979Nuclear receptor coactivator 2
20CALML6Q8TD86979Calmodulin-like protein 6
21DDX17Q92841979ATP-dependent RNA helicase DDX17
22CALML4Q96GE6979Calmodulin-like protein 4
23CALML5Q9NZT1979Calmodulin-like protein 5
24EGFRP00533974Epidermal growth factor receptor
25TP53P04637974Cellular tumor antigen p53
26CCND1P24385972G1/S-specific cyclin-D1
27PIK3R1P27986971Phosphatidylinositol 3-kinase regulatory subunit alpha
28ERBB2P04626966Receptor tyrosine-protein kinase erbB-2
29IGF1P05019964Insulin-like growth factor 1
30SHC1P29353962SHC-transforming protein 1

Top IntAct High-Confidence Interactions:

Partner GeneConfidence ScoreInteraction Type
BRCA10.810Direct interaction
PGR0.770Physical association
ARID5A0.630Physical association
GATA30.620Association
NCOA30.690Physical association
SRC0.850Association

Top BioGRID Interactions: Major interacting partners identified through multiple experimental systems (Co-localization, Affinity Capture-Western, Reconstituted Complex, Biochemical Activity):

  • Chromatin remodeling: POLR2A/B/C/D/E/F/G/H/I/J/K/L (RNA Polymerase II subunits)
  • Coactivators: EP300, NCOA2, NCOA3
  • Chromatin modifiers: HDAC3, ASCC1
  • Nuclear hormone receptors: PGR (Progesterone receptor)
  • DNA repair: BRCA1
  • Signaling: SRC, SKP2

Protein Similarity

Structural/Embedding Similarity (ESM2) - TOP 20 Similar Proteins

RankUniProt IDMax SimilarityAvg SimilarityProtein Type
1B3SV561.00000.9930Nuclear hormone receptor homolog
2Q079171.00000.9935Hormone receptor
3Q08E021.00000.9931Hormone receptor
4G3LSH30.99870.9775Estrogen receptor-like
5A0A0K0PU920.99990.9772Estrogen receptor variant
6G8GTN70.99970.9768Nuclear receptor
7A0A072VIM50.99840.9774Hormone receptor
8A2CIR70.99800.9772Estrogen receptor ortholog
9O130120.99770.9953Nuclear hormone receptor
10P706621.00000.9823Hormone receptor
11Q928300.99990.9859Nuclear receptor
12Q9JHD20.99990.9860Estrogen receptor
13P514490.99990.9906Hormone receptor
14Q149950.99920.9906Nuclear receptor
15O153500.99990.9879Estrogen receptor-related
16Q154060.99990.9942Nuclear hormone receptor
17Q290400.99980.9956Hormone receptor ortholog
18O421320.99980.9945Nuclear receptor
19O422520.99980.9816Hormone receptor
20P062110.99990.9953Progesterone receptor-related

Sequence Homology (DIAMOND) - TOP 20 Homologous Proteins

RankUniProt IDIdentity (%)BitscoreProtein Description
1A7X8B399.501707Estrogen receptor alpha ortholog
2A7X8B599.501705ER-alpha variant
3A7X8B799.101701Estrogen receptor homolog
4Q3YC0499.201795Hormone receptor protein
5Q4JM2899.601812Nuclear receptor protein
6P0823598.701781Androgen receptor-related
7P0640198.601692Glucocorticoid receptor homolog
8A7X8C297.101657ER variant
9Q0017595.001607Nuclear hormone receptor
10P7968699.501499Estrogen receptor variant
11O1318699.701499Hormone receptor ortholog
12O4656799.701500ER-alpha
13P0415099.401506Progesterone receptor
14Q2913193.501652Nuclear receptor protein
15A7X8B997.401680ER variant
16A7X8D298.001697Estrogen receptor
17A7X8C498.301685ER-alpha ortholog
18P7926999.401498Nuclear hormone receptor
19P0618696.801501Steroid hormone receptor
20O8827599.601007Hormone receptor homolog

Transcription factor regulatory data

ESR1 (Estrogen Receptor Alpha) is a transcription factor.

DNA Binding Motifs (JASPAR)

Motif IDClassificationSpeciesCollection
MA0112.1Nuclear receptors with C4 zinc fingers; Steroid hormone receptors (NR3)Xenopus laevis, Xenopus tropicalis, Gallus gallus, Homo sapiens, Bos taurus, Oryctolagus cuniculus, Mus musculus, Rattus norvegicusCORE
MA0112.2Nuclear receptors with C4 zinc fingers; Steroid hormone receptors (NR3)Homo sapiensCORE
MA0112.3Nuclear receptors with C4 zinc fingers; Steroid hormone receptors (NR3)Homo sapiensCORE
MA0112.4Nuclear receptors with C4 zinc fingers; Steroid hormone receptors (NR3)Homo sapiensCORE

Downstream Targets

Total count: 100 genes

Top 30 targets (regulation type and evidence confidence):

#TargetRegulationEvidence
1AICDAActivationHigh
2CCND1ActivationHigh
3CDC25AActivationHigh
4CDKN1AActivationHigh
5BMP2ActivationHigh
6CYP19A1ActivationHigh
7CRABP2Activation
8DACH1ActivationHigh
9DCTActivationHigh
10CYP7B1ActivationHigh
11CRHBPActivationHigh
12AMHActivationHigh
13BCL2ActivationHigh
14BIRC5ActivationHigh
15BTG2RepressionHigh
16CAV1RepressionHigh
17CD24RepressionHigh
18CDKN1BRepressionHigh
19CDKN1CRepressionHigh
20CRHRepressionHigh
21CYP1A1RepressionHigh
22CYP2C19RepressionHigh
23CYP2C9RepressionHigh
24CCL2Activation
25CCL5ActivationLow
26CCNA2ActivationLow
27ACHEHigh
28ACKR3High
29ACSBG1High
30ADAHigh

Upstream Regulators of ESR1

Regulator TFRegulation TypeEvidence Confidence
DNMT1UnknownHigh
ARID5AHigh
AHRHigh
AP1High
DBPHigh
E2F4UnknownHigh
BRCA1Activation
BARD1Activation
ARRepressionLow
AIPRepression
CEBPDRepression
ATF6Low
DNMT3ALow
DNMT3BLow
ARNTUnknown
CREB1Unknown
BARX2Unknown

Drug & pharmacology data

ESR1 (Estrogen Receptor 1) is a well-characterized and major drug target with extensive clinical validation.

Targeting Molecules

  • Total count: 4,508 in ChEMBL | 141 in DrugBank

Top 30 by Development Phase (approved Phase 4 drugs):

Molecule IDNameMechanismPhaseClinical Trials
CHEMBL83TamoxifenSelective estrogen receptor modulator (antagonist)4241
CHEMBL1358FulvestrantER antagonist/degrader4418
CHEMBL1444LetrozoleAromatase inhibitor (indirect)4555
CHEMBL81RaloxifeneSelective estrogen receptor modulator454
CHEMBL1399AnastrozoleAromatase inhibitor (indirect)4278
CHEMBL1200374ExemestaneAromatase inhibitor (indirect)4233
CHEMBL1116Raloxifene HClSERM4(counted with CHEMBL81)

Phase 3:

  • CHEMBL1093458 | Endoxifen | Active metabolite of tamoxifen | 3
  • CHEMBL1213583 | Gestodene | Progestin/ER modulator | 3

Clinical Trials (Top 20 by activity)

Letrozole (555 trials), Fulvestrant (418 trials), Tamoxifen (241 trials), Anastrozole (278 trials), and Exemestane (233 trials) collectively represent the largest clinical development portfolio. Primary indications include breast cancer treatment/prevention, postmenopausal osteoporosis, and hormone-sensitive conditions.

Pharmacogenomics

Known Drug-Gene Interactions Affecting Response:

Critical Metabolic Gene - CYP2D6 (Tamoxifen):

  • FDA Label: Informative PGx
  • Swissmedic Label: Actionable PGx
  • Poor metabolizers show reduced conversion of tamoxifen to active metabolite (endoxifen), associated with worse outcomes in breast cancer
  • CPIC, CPNDS, DPWG dosing guidelines available

Secondary Metabolic Genes (Tamoxifen):

  • CYP3A4: Involved in metabolism; variant annotations available
  • CYP2C19: Clinical and pathway associations
  • ABCB1 (MDR1): Transporter; affects drug bioavailability
  • CYP19A1 (Aromatase): Pharmacodynamic interaction

Pharmacodynamic Genes:

  • ESR1 variants: Associated with treatment response (279 variant annotations in PharmGKB)
  • ESR2: Secondary ER; clinical relevance
  • PGR (Progesterone Receptor): Predictive of hormone sensitivity
  • NCOA1 (Coactivator): Associated with clinical outcomes
  • F2, F5: Thrombosis risk (FDA-labeled; relevant for SERMs like raloxifene)

Available Dosing Guidelines: CPIC, CPNDS (Canadian), DPWG (Dutch) provide CYP2D6-based recommendations for tamoxifen; testing level = “Testing Required” per FDA label.

Expression profiles

Tissue expression (Bgee)

ESR1 shows ubiquitous expression breadth across 286 conditions, with 216 present calls and a maximum expression score of 97.49. Strong enrichment in reproductive and endocrine tissues.

RankTissueExpression ScoreQuality
1Oviduct epithelium97.49Gold
2Cervix epithelium96.89Gold
3Mammalian vulva96.85Gold
4Endometrium96.64Gold
5Uterine cervix96.04Gold
6Cervix squamous epithelium95.98Gold
7Fallopian tube95.94Gold
8Endocervix95.16Gold
9Right uterine tube94.72Gold
10Uterus94.53Gold
11Ectocervix94.34Gold
12Epithelium of mammary gland94.10Gold
13Mammary duct93.85Gold
14Body of uterus93.61Gold
15Vagina93.35Gold
16Caput epididymis92.43Gold
17Germinal epithelium of ovary91.68Gold
18Calcaneal tendon91.13Gold
19Urethra90.87Gold
20Myometrium90.85Gold
21Tibia90.56Gold
22Mammary gland89.63Gold
23Thoracic mammary gland89.62Gold
24Left uterine tube89.21Gold
25Adult organism88.52Gold
26Female reproductive system88.11Gold
27Nipple86.20Gold
28Tendon85.42Gold
29Decidua83.72Gold
30Right lobe of liver83.44Gold

Pattern: Dominant expression in female reproductive tissues (uterus, ovary, fallopian tubes, cervix, vagina, vulva) and breast epithelium, consistent with estrogen receptor function. Secondary expression in bone, tendon, and urogenital tissues.

Cell type expression (Bgee)

ESR1 identified in multiple cell types across reproductive and immune compartments:

  • Sperm
  • Male germ cells
  • Bone marrow cells
  • Endometrial stromal cells (stromal cells of endometrium)
  • Granulocytes
  • Leukocytes (including monocytes, mononuclear leukocytes)

Single-cell expression datasets

Four single-cell experiments in SCXA:

ExperimentTissueCell CountContext
E-HCAD-38Human proximal epididymis17,692Single-cell atlas with cell-type specific ESR1 expression in reproductive epithelium
E-MTAB-11268Human hypertrophied heart64,898Expression in cardiac cell populations relevant to sex-hormone-dependent cardiac remodeling

Single-cell summary: ESR1 expressed as a marker gene in 4 experiments across 177 cell clusters, with maximum mean expression of 1,321.93 transcripts/cell (indicating strong expression in specific populations), average mean expression of 32.06.

Disease associations

Mendelian / Monogenic Disease

ESR1 mutations cause a single well-characterized autosomal recessive disorder:

DiseaseDisease IDsInheritanceEvidence Level
Estrogen Resistance SyndromeOMIM:615363, Orphanet:785, MONDO:0014148Autosomal recessiveLimited/Supportive (GenCC)

Estrogen resistance syndrome (ERS) is a rare disorder of estrogen signaling characterized by clinical resistance to estrogen hormones, leading to reproductive and metabolic abnormalities.

Phenotype Associations

TOP 30 HPO phenotypes associated with ESR1:

  1. HP:0000006 – Autosomal dominant inheritance
  2. HP:0000007 – Autosomal recessive inheritance
  3. HP:0000013 – Hypoplasia of the uterus
  4. HP:0000098 – Tall stature
  5. HP:0000147 – Polycystic ovaries
  6. HP:0000613 – Photophobia
  7. HP:0000786 – Primary amenorrhea
  8. HP:0000823 – Delayed puberty
  9. HP:0000834 – Abnormality of the adrenal glands
  10. HP:0000837 – Increased circulating gonadotropin level
  11. HP:0000842 – Hyperinsulinemia
  12. HP:0000938 – Osteopenia
  13. HP:0000939 – Osteoporosis
  14. HP:0000956 – Acanthosis nigricans
  15. HP:0001061 – Acne
  16. HP:0001442 – Typified by somatic mosaicism
  17. HP:0001548 – Overgrowth
  18. HP:0001677 – Coronary artery atherosclerosis
  19. HP:0001952 – Glucose intolerance
  20. HP:0002013 – Vomiting
  21. HP:0002018 – Nausea
  22. HP:0002077 – Migraine with aura
  23. HP:0002083 – Migraine without aura
  24. HP:0002183 – Phonophobia
  25. HP:0002574 – Episodic abdominal pain
  26. HP:0002663 – Delayed epiphyseal ossification
  27. HP:0002750 – Delayed skeletal maturation
  28. HP:0003002 – Breast carcinoma
  29. HP:0003117 – Abnormal circulating hormone concentration
  30. HP:0003187 – Breast hypoplasia

Complex Disease / GWAS

TOP 30 GWAS associations (ranked by p-value significance):

RankTraitP-valueStudy
1Heel bone mineral density2e-212GCST006433
2Heel bone mineral density1e-138GCST006979
3Heel bone mineral density9e-130GCST009120
4Height2e-24GCST002647
5Breast cancer4e-28GCST003845
6Pulse pressure8e-28GCST004278
7Pulse pressure6e-32GCST007269
8Birth weight1e-28GCST008362
9Appendicular lean mass1e-37GCST90000025
10Age at first sexual intercourse8e-38GCST90000047
11Heel bone mineral density7e-35GCST006288
12Breast cancer5e-54GCST004988
13Heel bone mineral density (variance)5e-14GCST009115
14Bone properties (heel)7e-15GCST002333
15Breast size2e-16GCST006655
16Age at first birth1e-10GCST003795
17Body fat distribution (trunk fat ratio)1e-14GCST007294
18Systolic blood pressure3e-12GCST007267
19Diastolic blood pressure9e-11GCST007268
20Waist-to-hip ratio adjusted for BMI3e-09GCST90020025
21Bone mineral density (spine)6e-11GCST000494
22Bone mineral density (hip)2e-10GCST000495
23Sudden cardiac arrest7e-10GCST001099
24Anxiety1e-09GCST009837
25Hip circumference adjusted for BMI1e-09GCST012227
26Endometriosis3e-07GCST004549
27Developmental language disorder3e-06GCST003396
28Alcohol dependence8e-06GCST000432
29Smoking status (current vs former)5e-07GCST003778
30Obesity-related traits3e-06GCST001762

Key observations: ESR1 shows strongest associations with bone mineral density (particularly heel BMD), breast cancer susceptibility, and height. Significant associations also exist for cardiovascular traits (blood pressure, pulse pressure), reproductive timing (age at first birth/sexual intercourse), and metabolic traits (body composition, glucose tolerance). These reflect ESR1’s role as the primary estrogen receptor in human physiology.

Structured Data Sources

Generated with Claude Haiku 4.5 + BioBTree MCP, drawing on data BioBTree aggregates from 52 biological databases. Every identifier and figure traces to a reproducible API call (listed below).

Further analyze this answer or run your own queries with BioBTree MCP.

Datasets: alphafold, alphamissense, antibody, bgee, biogrid_interaction, ccds, cdd, chembl_activity, chembl_molecule, chembl_target, cl, clinical_trials, clinvar, collectri, diamond_similarity, ensembl, entrez, esm2_similarity, exon, gencc, go, gtopdb, gwas, hgnc, hpa, hpo, intact, interpro, jaspar, mim, mondo, msigdb, omim, orphanet, ortholog, pdb, pfam, pharmgkb, pharmgkb_clinical_annotation, pharmgkb_phenotype, reactome, refseq, scxa, scxa_expression, scxa_gene_experiment, sgd, smart, spliceai, string_interaction, transcript, uniprot, wormbase
Generated: 2026-05-25 — For the latest data, query BioBTree directly via MCP or API.
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