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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
VAX1
ventral anterior homeobox 1
Chromosome 10 Β· 10q25.3
NCBI Gene: 11023Ensembl: ENSG00000148704.13HGNC: HGNC:12660UniProt: Q5SQQ9
27PubMed Papers
21Diseases
0Drugs
2Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
sequence-specific double-stranded DNA bindingregulation of transcription by RNA polymerase IIcentral nervous system developmentbrain developmentmicrophthalmiasubstance-related disordercleft lipAbruptio Placentae
✦AI Summary

VAX1 is a homeodomain transcription factor that functions as a sequence-specific DNA-binding regulator of gene expression 1. Developmentally, VAX1 is required for dorsoventral specification of the forebrain, axon guidance, and major tract formation [UniProt]. VAX1 plays critical roles in suprachiasmatic nucleus (SCN) development and function, including regulation of vasoactive intestinal peptide expression and circadian output 1. The gene is also essential for gonadotropin-releasing hormone (GnRH) neuron development; heterozygous VAX1 deletion causes subfertility through hypothalamic dysfunction, with reduced GnRH neuron numbers and altered kisspeptin expression 2. VAX1 contributes to neuroretinal and optic development, with homozygous mutations causing microphthalmia, small optic nerves, and corpus callosum agenesis 3. Clinically, VAX1 variants are strongly associated with nonsyndromic cleft lip with or without palate (NSCL/P) across multiple populations 456. Common variants (rs7078160, rs4752028) show significant over-transmission in affected families, with both maternal and paternal contributions 46. Multiple rare missense mutations in VAX1 have been identified in NSCL/P patients, though penetrance appears incomplete 76. VAX1 mutations are also associated with syndromic microphthalmia 3. The gene represents an important susceptibility locus for craniofacial development and reproductive function.

Sources cited
1
VAX1 is required for SCN development, vasoactive intestinal peptide expression, and SCN output function
PMID: 31705443
2
Heterozygous VAX1 deletion causes subfertility through hypothalamic effects on GnRH neuron development and number
PMID: 25060364
3
VAX1 homozygous mutations cause microphthalmia, small optic nerves, cleft lip/palate, and corpus callosum agenesis
PMID: 22095910
4
VAX1 rs7078160 allele A is a risk factor for NSCL/P with paternal over-transmission in Western Han Chinese
PMID: 30048854
5
Six SNPs in VAX1 are associated with NSCL/P; five influence transcription factor-binding ability and act as eQTLs
PMID: 35419918
6
VAX1 markers rs7078160 and rs4752028 show strong associations with NSCL/P in Mongolian and Japanese populations with both maternal and paternal transmission effects
PMID: 23463464
7
Rare variants in VAX1 are identified in NSCL/P patients but show incomplete cosegregation in families
PMID: 23081944
Disease Associationsβ“˜21
microphthalmiaOpen Targets
0.59Moderate
substance-related disorderOpen Targets
0.39Weak
cleft lipOpen Targets
0.39Weak
Abruptio PlacentaeOpen Targets
0.32Weak
cleft palateOpen Targets
0.30Weak
orofacial cleftOpen Targets
0.25Weak
mathematical abilityOpen Targets
0.24Weak
obesityOpen Targets
0.16Weak
overnutritionOpen Targets
0.13Weak
smoking initiationOpen Targets
0.06Suggestive
holoprosencephalyOpen Targets
0.06Suggestive
catecholaminergic polymorphic ventricular tachycardiaOpen Targets
0.06Suggestive
diabetes mellitusOpen Targets
0.05Suggestive
Arrhythmogenic right ventricular dysplasiaOpen Targets
0.05Suggestive
Romano-Ward syndromeOpen Targets
0.05Suggestive
Familial short QT syndromeOpen Targets
0.05Suggestive
coloboma of maculaOpen Targets
0.05Suggestive
holoprosencephaly 9Open Targets
0.05Suggestive
OligodontiaOpen Targets
0.04Suggestive
tooth agenesisOpen Targets
0.04Suggestive
Microphthalmia, syndromic, 11UniProt
Pathogenic Variants2
NM_001112704.2(VAX1):c.212del (p.Pro71fs)Likely pathogenic
Microphthalmia
β˜…β˜†β˜†β˜†2022β†’ Residue 71
NM_001112704.2(VAX1):c.454C>A (p.Arg152Ser)Pathogenic
Microphthalmia, syndromic 11
β˜†β˜†β˜†β˜†2012β†’ Residue 152
View on ClinVar β†—
Related Genes
EMX1Shared pathway100%EMX2Shared pathway100%NOTOShared pathway100%GSX1Shared pathway100%ASXL2Protein interaction92%ZNF462Protein interaction90%
Tissue Expression6 tissues
Brain
100%
Bone Marrow
9%
Heart
0%
Ovary
0%
Liver
0%
Lung
0%
Gene Interaction Network
Click a node to explore
VAX1EMX1EMX2NOTOGSX1ASXL2ZNF462
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q5SQQ9
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
0.68LoF Tolerant
pLIβ“˜
0.66Intermediate
Observed/Expected LoF0.34 [0.19–0.68]
RankingsWhere VAX1 stands among ~20K protein-coding genes
  • #12,705of 20,598
    Most Researched27
  • #4,529of 5,498
    Most Pathogenic Variants2
  • #5,037of 17,882
    Most Constrained (LOEUF)0.68
Genes detectedVAX1
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
The Homeodomain Transcription Factors Vax1 and Six6 Are Required for SCN Development and Function.
PMID: 31705443
Mol Neurobiol Β· 2020
1.00
2
Target sequencing reveals the association between variants in VAX1 and NSCL/P in Chinese population.
PMID: 35419918
Oral Dis Β· 2023
0.90
3
VAX1 gene associated non-syndromic cleft lip with or without palate in Western Han Chinese.
PMID: 30048854
Arch Oral Biol Β· 2018
0.80
4
VAX1 mutation associated with microphthalmia, corpus callosum agenesis, and orofacial clefting: the first description of a VAX1 phenotype in humans.
PMID: 22095910
Hum Mutat Β· 2012
0.70
5
Heterozygous deletion of ventral anterior homeobox (vax1) causes subfertility in mice.
PMID: 25060364
Endocrinology Β· 2014
0.60