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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
ESX1
ESX homeobox 1
Chromosome X Β· Xq22.2
NCBI Gene: 80712Ensembl: ENSG00000123576.5HGNC: HGNC:14865UniProt: Q8N693
22PubMed Papers
20Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
DNA-binding transcription repressor activity, RNA polymerase II-specificregulation of cell cyclenucleusnuclear speckmale infertility with azoospermia or oligozoospermia due to single gene mutationneurodegenerative diseaseinfectionplacental insufficiency
✦AI Summary

ESX1 is an X-linked homeobox gene encoding a transcription factor with dual roles in spermatogenesis and placental development. Functionally, ESX1 inhibits polyubiquitinated cyclin A and cyclin B1 degradation, arresting the cell cycle at early M phase during male germ cell division 1. The N-terminal region acts as a transcriptional repressor binding to specific DNA sequences (5'-TAATGTTATTA-3'), including within the KRAS gene promoter, while the C-terminal region regulates cyclin stability 1. ESX1 expression serves as a robust molecular marker for residual spermatogenesis; its detection in testicular tissue correlates strongly with successful sperm recovery in azoospermic patients, with lower promoter methylation associated with expression 2. Clinically, mutations in ESX1 are associated with non-obstructive azoospermia (NOA) in multiple populations; specific variants like the compound mutation p.[P365R; L366V] impair cyclin stabilization, compromising M phase arrest and contributing to male infertility 34. ESX1 activation in germ cells regulates networks of genes involved in cell proliferation and differentiation, with altered expression patterns observed in azoospermic patients carrying ESX1 mutations 4. Additionally, ESX1 shows evidence of positive selection in primate evolution, particularly in the C-terminal region, suggesting reproductive significance 1.

Sources cited
1
ESX1 is X-linked, expressed in testis and placenta, regulates cyclin stability in C-terminal region, shows positive selection in primate evolution
PMID: 17588961
2
ESX1 transcript detection correlates with successful sperm recovery in azoospermic patients; lower methylation in expressors
PMID: 20356899
3
ESX1 mutations, particularly p.P365R, impair cyclin A stabilization and cause M phase arrest failure in NOA
PMID: 33633269
4
ESX1 activation regulates >50 genes involved in cell proliferation/differentiation; inverse expression correlations in azoospermic patients with ESX1 mutations
PMID: 37783880
⚠Limited data available β€” This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsβ“˜20
male infertility with azoospermia or oligozoospermia due to single gene mutationOpen Targets
0.27Weak
neurodegenerative diseaseOpen Targets
0.22Weak
infectionOpen Targets
0.05Suggestive
placental insufficiencyOpen Targets
0.04Suggestive
osteoarthritis, hipOpen Targets
0.04Suggestive
fetal growth restrictionOpen Targets
0.03Suggestive
childhood supratentorial ependymomaOpen Targets
0.02Suggestive
GranulomaOpen Targets
0.02Suggestive
azoospermiaOpen Targets
0.01Suggestive
Townes-Brocks syndromeOpen Targets
0.01Suggestive
ependymomaOpen Targets
0.01Suggestive
male infertilityOpen Targets
0.01Suggestive
Duane retraction syndromeOpen Targets
0.01Suggestive
tuberculosisOpen Targets
0.01Suggestive
Sertoli Cell-Only SyndromeOpen Targets
0.00Suggestive
gliomaOpen Targets
0.00Suggestive
SubependymomaOpen Targets
0.00Suggestive
cancerOpen Targets
0.00Suggestive
neoplasmOpen Targets
0.00Suggestive
testicular seminomaOpen Targets
0.00Suggestive
Pathogenic Variants1
NM_153448.4(ESX1):c.1094C>G (p.Pro365Arg)Likely pathogenic
Male infertility with azoospermia or oligozoospermia due to single gene mutation
β˜…β˜†β˜†β˜†2023β†’ Residue 365
View on ClinVar β†—
Related Genes
PDE6BProtein interaction87%POU1F1Protein interaction84%SOX3Protein interaction84%LHX3Protein interaction84%LHX4Protein interaction84%ELF3Protein interaction77%
Tissue Expression6 tissues
Lung
0%
Brain
0%
Heart
0%
Ovary
0%
Bone Marrow
0%
Liver
0%
Gene Interaction Network
Click a node to explore
ESX1PDE6BPOU1F1SOX3LHX3LHX4ELF3
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q8N693
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.48Moderately Constrained
pLIβ“˜
0.97Intolerant
Observed/Expected LoF0.15 [0.06–0.48]
RankingsWhere ESX1 stands among ~20K protein-coding genes
  • #13,658of 20,598
    Most Researched22
  • #5,348of 5,498
    Most Pathogenic Variants1
  • #2,774of 17,882
    Most Constrained (LOEUF)0.48 Β· top quartile
Genes detectedESX1
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
ESX1 gene as a potential candidate responsible for male infertility in nonobstructive azoospermia.
PMID: 37783880
Sci Rep Β· 2023
1.00
2
Rapid evolution of primate ESX1, an X-linked placenta- and testis-expressed homeobox gene.
PMID: 17588961
Hum Mol Genet Β· 2007
0.90
3
Whole-genome sequencing identifies new candidate genes for nonobstructive azoospermia.
PMID: 36017582
Andrology Β· 2022
0.80
4
Association of ESX1 gene variants with non-obstructive azoospermia in Chinese males.
PMID: 33633269
Sci Rep Β· 2021
0.70
5
Immunopeptidomics reveals determinants of
PMID: 37073954
Elife Β· 2023
0.60