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Symbol EN1 contributors: mct - updated : 27-03-2019
HGNC name engrailed homeobox 1
HGNC id 3342
Location 2q14.2      Physical location : 119.599.747 - 119.605.759
Synonym name engrailed homolog 1
TYPE functioning gene
STRUCTURE 5.03 kb     2 Exon(s)
10 Kb 5' upstream gene genomic sequence study
MAPPING cloned Y linked   status confirmed
Physical map
DPP10 2q14.1 dipeptidylpeptidase 10 LOC391430 2 similar to laminin receptor-like protein LAMRL5 DDX18 2q13 DEAD (Asp-Glu-Ala-Asp) box polypeptide 18 LOC389024 2 LOC389024 LOC343958 2q14.2 similar to 5-HT5B serotonin receptor FLJ10996 2q14.2 hypothetical protein FLJ10996 INSIG2 2q21.2 insulin induced gene 2 LOC389025 2 LOC389025 LOC151154 2q14.2 hypothetical LOC151154 EN1 2q14.2 engrailed homolog 1 MARCO 2q14.2 macrophage receptor with collagenous structure LOC165257 2q14.2 C1q-domain containing protein TSAP6 2q14.2 C1q-domain containing protein LOC389026 2 similar to PRO1546 DBI 2q12-q21 diazepam binding inhibitor (GABA receptor modulator, acyl-Coenzyme A binding protein) PR1 2q14.2 voltage-dependent calcium channel gamma subunit-like protein SCTR 2q14.1 secretin receptor LOC200373 2q14.2 similar to hypothetical protein MGC10993 2q14.2 hypothetical protein MGC10993 PTPN4 2p24.3-p24.1 protein tyrosine phosphatase, non-receptor type 4 (megakaryocyte) EPB41L5 2q21.2 erythrocyte membrane protein band 4.1 like 5 LOC391431 2 similar to NADH dehydrogenase subunit 5 FAM11B 2q21.2 similar to NADH dehydrogenase subunit 5 RALB 2cen-q13 v-ral simian leukemia viral oncogene homolog B (ras related; GTP binding protein) INHBB 2q12-q13 inhibin, beta B (activin AB beta polypeptide) FLJ14816 2q14.2 hypothetical protein FLJ14816 GLI2 2q24 GLI-Kruppel family member GLI2 TFCP2L1 2q14 transcription factor CP2-like 1 CLASP1 2q14-q21 cytoplasmic linker associated protein 1 MKI67IP 2q14.3 MKI67 (FHA domain) interacting nucleolar phosphoprotein TSN 2q21.1 translin LOC389027 2 LOC389027 LOC389028 2 LOC389028
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
2 - 2962 - 392 - 2014 24685177
Type restricted
   expressed in (based on citations)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Nervousbrainhindbraincerebellum   Mus musculusFetal
Reproductivefemale systemplacenta  lowly
Respiratorylung   lowly
Skin/Tegumentskin appendagesskin gland    Homo sapiens
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Connectivebone  lowly
Epithelialsecretoryglandularexocrine  Homo sapiens
SystemCellPubmedSpeciesStageRna symbol
Nervousneuron Mus musculusFetal
cell lineage
cell lines
physiological period embryo, pregnancy
Text pregnancy, embryo, ventral limb ectoderm
  • an eukaryotic translation initiation factor 4E (eIF4E) binding domain
  • five EN homology regions (EH)
  • a Groucho binding domain
  • two PBX binding domains
  • an helix-turn-helix DNA binding domain
  • a cell penetration domain
  • a C-terminal transcriptional repression domain
    interspecies homolog to murine En1 (95.10 pc)
    homolog to drosophila en (70.00 pc)
    intraspecies paralog to EN2 gene
    FAMILY engrailed homeobox family
    CATEGORY regulatory , transcription factor
    SUBCELLULAR LOCALIZATION     intracellular
    basic FUNCTION
  • regulator of the ventral limb patterning
  • inhibiting WNT7A expression in ventral limb bud
  • involved in transcriptional and translational modulation
  • seems to be capable of acting as a morphogen in its own right
  • EN1, EN2 represent a new class of genes that are fundamental to patterning cerebellum foliation throughout the mediolateral axis and that act late in development
  • EN1, EN2 are required for patterning the morphological and molecular domains along both axes, as well as for the establishment of the normal afferent topography required to generate a fully functional cerebellum
  • functions upstream of FGFR2 in regulating early calvarial osteogenic differentiation, and likely it plays an additional role in precluding premature osteogenic conversion of the sutural mesenchyme
  • EN1 and EN2 have overlapping functions in the cells derived from each progenitor zone in regulating formation of additional fissures and for extensive cerebellar growth
  • is an activator of intrinsic inflammatory pathways associated with prosurvival in basal-like breast cancer
  • expressed in the ventral ectoderm of the developing limb where it plays an instructive role in the dorsal-ventral patterning of the forelimb (PMUID: 25710467)
  • EN1 but not EN2 has a direct and specific repulsive effect on motor axons of the lateral aspect of the lateral motor column (LMC) but not on medial LMC projections
  • important roles for EN1 in the development and maturation of a diverse group of brainstem auditory neurons
  • is a gene important for ventral nuclei of the lateral lemniscus (VNLL) neuron development and survival
  • regulated levels of PITX3 and EN1 control the size and rostral/caudal-identity of the Mesodiencephalic dopaminergic (mdDA) neuronal population
  • plays an important role in the regionalization of the neural tube
  • CELLULAR PROCESS nucleotide, transcription, regulation
    protein, translation regulation
    PHYSIOLOGICAL PROCESS development , nervous system
    a component
    DNA binding
    small molecule
  • binding
  • cell & other
    induced by FGF8
    Other regulated by NURF complex
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral   deletion    
    repression of the entire 4-Mb band of chromosome 2q.14.2, associated with global methylation of histone H3 Lys9 in colorectal cancer
    tumoral     --other  
    expressed focally in the epidermal neoplasms of seborrheic keratosis and squamous cell carcinoma
    causes mispatterning of the forelimb and thus defects in the dorsal-ventral pathfinding choice of motor axons
    Variant & Polymorphism
    Candidate gene
    Therapy target
    En1 knockout mice