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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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TRERF1
transcriptional regulating factor 1
Chromosome 6 · 6p21.1
NCBI Gene: 55809Ensembl: ENSG00000124496.13HGNC: HGNC:18273UniProt: A0A8Q3SI57
40PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
transcription coactivator bindingtranscription coactivator activitypositive regulation of DNA-templated transcriptionpositive regulation of transcription by RNA polymerase IIAbnormality of the skeletal systemneurodegenerative diseasehemolytic anemiageneralized dystonia
✦AI Summary

TRERF1 (transcriptional regulating factor 1) is a nuclear DNA-binding transcription factor that plays a crucial role in steroid hormone regulation. TRERF1 activates transcription of CYP11A1, a key enzyme in steroid hormone synthesis, through direct DNA binding and synergistic interactions with coactivators CREBBP and EP300 1. The gene functions in both positive and negative regulation of RNA polymerase II-mediated transcription and participates in progesterone receptor signaling pathways. Clinically, TRERF1 has relevance across multiple disease contexts. Novel somatic mutations in TRERF1 (p.K1056Q and p.G1063R) have been identified in ovarian endometriosis patients, suggesting involvement in endometriosis pathogenesis through dysregulation of steroid hormone production 2. In breast cancer, TRERF1 was identified as a BCAR (breast cancer antiestrogen resistance) gene conferring tamoxifen resistance and estrogen independence; elevated TRERF1 expression associates with reduced metastasis-free survival in lymph node-negative breast cancer patients, independent of traditional prognostic factors 1 3. TRERF1 expression is upregulated in class-switched memory B cells, suggesting roles in adaptive immunity 4. Additionally, TRERF1 variants have been identified in intellectual disability cohorts 5 and obesity-associated bladder cancer 6, indicating broader pathogenic potential across diverse conditions.

Sources cited
1
TRERF1/BCAR2 directly confers estrogen independence in breast cancer cells through functional genetic screening
PMID: 18351453
2
Novel TRERF1 somatic mutations (p.K1056Q and p.G1063R) identified in ovarian endometriosis patients; TRERF1 has crucial role in steroid hormone production
PMID: 29393434
3
TRERF1 mRNA levels are significantly associated with metastasis-free survival in lymph node-negative breast cancer patients independent of traditional prognostic factors
PMID: 19075277
4
TRERF1 is upregulated in class-switched memory B cells as part of core transcriptional signature, with MIR181 regulation
PMID: 33116135
5
TRERF1 variants identified as potential candidate gene in intellectual disability cohort through exome sequencing
PMID: 27431290
6
TRERF1 is among prioritized genes significantly associated with BMI in bladder urothelial cancer
PMID: 41872334
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.38Weak
neurodegenerative diseaseOpen Targets
0.28Weak
hemolytic anemiaOpen Targets
0.25Weak
generalized dystoniaOpen Targets
0.24Weak
diabetes mellitusOpen Targets
0.22Weak
metabolic syndrome XOpen Targets
0.21Weak
Lower limb asymmetryOpen Targets
0.21Weak
systemic lupus erythematosusOpen Targets
0.08Suggestive
experimental autoimmune encephalomyelitisOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.08Suggestive
cardiac hypertrophyOpen Targets
0.06Suggestive
acute myeloid leukemiaOpen Targets
0.06Suggestive
Alzheimer diseaseOpen Targets
0.05Suggestive
non-small cell lung carcinomaOpen Targets
0.04Suggestive
osteosarcomaOpen Targets
0.04Suggestive
hepatocellular carcinomaOpen Targets
0.03Suggestive
graft versus host diseaseOpen Targets
0.02Suggestive
gliomaOpen Targets
0.02Suggestive
infectionOpen Targets
0.02Suggestive
cancerOpen Targets
0.02Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
DNTTIP1Protein interaction100%HDAC1Protein interaction97%MIDEASProtein interaction87%NR5A1Protein interaction77%WBP2Shared pathway13%PGRShared pathway11%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
28%
Brain
13%
Lung
12%
Heart
10%
Liver
4%
Gene Interaction Network
Click a node to explore
TRERF1DNTTIP1HDAC1MIDEASNR5A1WBP2PGR
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q05GC8
View on AlphaFold ↗
RankingsWhere TRERF1 stands among ~20K protein-coding genes
  • #10,257of 20,598
    Most Researched40
Genes detectedTRERF1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Novel TRERF1 mutations in Chinese patients with ovarian endometriosis.
PMID: 29393434
Mol Med Rep · 2018
1.00
2
Integrative transcriptome and chromatin landscape analysis reveals distinct epigenetic regulations in human memory B cells.
PMID: 33116135
Nat Commun · 2020
0.90
3
Functional identification of genes causing estrogen independence of human breast cancer cells.
PMID: 18351453
Breast Cancer Res Treat · 2009
0.80
4
Novel candidate genes for ECT response prediction-a pilot study analyzing the DNA methylome of depressed patients receiving electroconvulsive therapy.
PMID: 32727556
Clin Epigenetics · 2020
0.70
5
Clinical genomics expands the morbid genome of intellectual disability and offers a high diagnostic yield.
PMID: 27431290
Mol Psychiatry · 2017
0.60