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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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EN2
engrailed homeobox 2
Chromosome 7 · 7q36.3
NCBI Gene: 2020Ensembl: ENSG00000164778.4HGNC: HGNC:3343UniProt: P19622
69PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
sequence-specific double-stranded DNA bindingnucleolusfibrillar centernucleoplasmviral pneumoniaProptosisprostatitisovarian neoplasm
✦AI Summary

EN2 (engrailed homeobox 2) is a homeodomain-containing transcription factor that functions as both a sequence-specific DNA-binding protein and a secreted signaling molecule 1. As a transcription factor, EN2 exhibits dual regulatory activity, functioning as both a transcriptional activator and repressor depending on cellular context, with the ability to bind RNA polymerase II cis-regulatory regions and modulate gene expression 23. EN2 demonstrates unique intercellular mobility, capable of traveling between cells and modifying transcriptional activity in recipient cells 4. During development, EN2 is strongly expressed in rhombic lip derivatives including cerebellar structures, inferior olive, and arcuate nucleus, suggesting roles in central nervous system patterning 5. In cancer contexts, EN2 acts as an oncogene, promoting tumor progression through multiple mechanisms including direct transcriptional regulation of target genes like CCL20 in colorectal cancer 2 and stabilization of NOTCH pathway signaling in head and neck squamous cell carcinoma 3. EN2's secretion into urine by prostate cancer cells, but not normal prostate tissue, makes it a promising diagnostic biomarker 1. Additionally, EN2 hypermethylation serves as a prognostic marker in colorectal cancer 6.

Sources cited
1
EN2 is a homeodomain-containing transcription factor secreted by prostate cancer into urine, serving as a potential diagnostic biomarker
PMID: 26411411
2
EN2 acts as an oncogene in colorectal cancer, directly regulating CCL20 expression and promoting tumor progression
PMID: 32732864
3
EN2 stabilizes NOTCH pathway signaling in head and neck squamous cell carcinoma progression
PMID: 41419462
4
EN2 is a traveling homeoprotein capable of intercellular mobility and modifying transcriptional activity in recipient cells
PMID: 40760093
5
EN2 is strongly expressed in rhombic lip derivatives including cerebellar structures during human fetal development
PMID: 9056537
6
EN2 hypermethylation serves as a prognostic marker in colorectal cancer
PMID: 35169223
Disease Associationsⓘ20
viral pneumoniaOpen Targets
0.13Weak
ProptosisOpen Targets
0.12Weak
prostatitisOpen Targets
0.10Weak
ovarian neoplasmOpen Targets
0.10Weak
Genu valgumOpen Targets
0.09Suggestive
Genu varumOpen Targets
0.09Suggestive
breast cancerOpen Targets
0.09Suggestive
prostate cancerOpen Targets
0.09Suggestive
posterior cortical atrophyOpen Targets
0.09Suggestive
colorectal carcinomaOpen Targets
0.09Suggestive
gliomaOpen Targets
0.09Suggestive
benign prostatic hyperplasiaOpen Targets
0.08Suggestive
pachyonychia congenitaOpen Targets
0.08Suggestive
Spinocerebellar ataxia type 41Open Targets
0.08Suggestive
neoplasmOpen Targets
0.08Suggestive
glioblastoma multiformeOpen Targets
0.08Suggestive
esophageal squamous cell carcinomaOpen Targets
0.08Suggestive
triple-negative breast cancerOpen Targets
0.07Suggestive
autismOpen Targets
0.07Suggestive
spinocerebellar ataxia type 30Open Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
VSTM2LShared pathway33%LHX4Shared pathway33%LHX5Shared pathway33%NIF3L1Shared pathway33%LHX9Shared pathway33%OTPShared pathway33%
Tissue Expression6 tissues
Ovary
100%
Brain
31%
Liver
3%
Lung
2%
Heart
0%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
EN2VSTM2LLHX4LHX5NIF3L1LHX9OTP
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt P19622
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.78LoF Tolerant
pLIⓘ
0.39Tolerant
Observed/Expected LoF0.39 [0.21–0.78]
RankingsWhere EN2 stands among ~20K protein-coding genes
  • #6,797of 20,598
    Most Researched69
  • #6,308of 17,882
    Most Constrained (LOEUF)0.78
Genes detectedEN2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
EN2 in Prostate Cancer.
PMID: 26411411
Adv Clin Chem · 2015
1.00
2
X Chromosome Contribution to the Genetic Architecture of Primary Biliary Cholangitis.
PMID: 33675743
Gastroenterology · 2021
0.90
3
Of travelling homeoproteins and medulloblastomas.
PMID: 40760093
Oncogene · 2025
0.80
4
A novel super-enhancer-driven lncRNA LINC00973 governs head and neck squamous cell carcinoma progression through EN2.
PMID: 41419462
Cell Death Dis · 2025
0.70
5
EN2 as an oncogene promotes tumor progression via regulating CCL20 in colorectal cancer.
PMID: 32732864
Cell Death Dis · 2020
0.60