Selected-GenAtlas references SOURCE GeneCards NCBI Gene Swiss-Prot Ensembl
HGNC UniGene Nucleotide OMIM UCSC
Home Page
Symbol NR2F2 contributors: mct/npt - updated : 25-09-2015
HGNC name nuclear receptor subfamily 2, group F, member 2
HGNC id 7976
Corresponding disease
DEL15QD chromosome 15q distal deletion
DIH1 congenital diaphragmatic hernia and 15q26.1-26.2 deletion
VCHD virilization and congenital heart disease (CHD)
Location 15q26.2      Physical location : 96.869.156 - 96.883.490
Synonym name
  • apolipoprotein regulatory protein 1
  • transcription factor COUP 2 (chicken ovalbumin upstream promoter 2,
  • apolipoprotein regulatory protein)
  • ADP-ribosylation factor related protein 1
  • apolipoprotein AI regulatory protein 1
  • chicken ovalbumin upstream promoter transcription factor II
  • Synonym symbol(s) ARP1, TFCOUP2, COUPTF2, SVP40, COUP-TFII, COUPTFB
    TYPE functioning gene
    STRUCTURE 14.34 kb     3 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    3 - 5110 - 414 - 2013 23458092
    3 - 3962 - 261 - 2013 23458092
    3 - 3501 - 261 - 2013 23458092
    3 - 3869 - 281 - 2013 23458092
  • variant 2
  • lacking a DNA-binding domain
  • negatively regulates the function of NR2F2 by inhibiting its binding to DNA to decrease CYP7A1 expression
    Type widely
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart   moderately
     vessels   moderately
    Endocrineadrenal gland    
    Lymphoid/Immunespleen   highly
    Reproductivefemale systemovary    Homo sapiens
     male systemtestis   
    Urinarybladder   highly
     kidney   highly
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    cell lineage
    cell lines
    at STAGE
  • a N terminal modulating domain
  • a central bipartite zinc finger, DNA binding domain
  • a C terminal ligand domain, strongly repressing NTS transcription, whereas the N-terminal domain antagonizes this repressive effect (MID: 21030593)
  • mono polymer homomer , dimer
    interspecies ortholog to rattus Nr2f2
    ortholog to murine Nr2f2
    homolog to C. elegans unc-55
  • small family of the steroid/thyroid hormone receptor nuclear superfamily of transcription factors
  • NR2 subfamily
  • CATEGORY regulatory , receptor nuclear membrane serine/threonine
    SUBCELLULAR LOCALIZATION     intracellular
    basic FUNCTION
  • may be required for angiogenesis and heart developpment
  • negative post transcriptional regulator of MYOD1 function
  • involved in the regulation of the apolipoprotein A-I gene transcription
  • playing a critical role in repressing Notch signaling to maintain vein identity
  • having an important maternal function in placental formation and involved in trophoblast giant cells differentiation
  • plays important roles in the regulation of the function of PROX1, and regulates the functions of PROX1 in lymphatic endothelial cells through direct interaction (Yamazaki 2009)
  • functions as a coregulator of PROX1 to control several lineage-specific genes including FLT4, FGFR3, and NRP1 and is required along with PROX1 to maintain lymphatic endothelial cells phenotype (Lee 2009)
  • directly regulates the transcription of angiopoietin-1 in pericytes to enhance neoangiogenesis (Qin 2010)
  • controls angiopoietin-1/Tie2 signaling to regulate tumor angiogenesis (Qin 2010)
  • important for both initiation and continued expansion and maintenance of angiogenic process (Qin 2010)
  • with NR2F1 play roles in amacrine cell differentiation (Inoue 2010)
  • involved in the organogenesis of the stomach, limbs, skeletal muscles, and heart
  • is required for the specification of the metanephric mesenchyme
  • plays a central role in the specification of metanephric fate and in the maintenance of metanephric mesenchyme proliferation and survival by acting as a crucial regulator of EYA1 and WT1 expression
  • pleiotropic developmental transcription factor
  • play a key role in integrating genetic and environmental risk factors for abnormal cardiac development
  • is a human "pro-ovary" and "anti-testis" sex-determining factor in female gonads
  • may be required to establish ovary identity during early human gonad development by the repression of genes involved in testis determination
  • acts both positively and negatively to modulate the expression of genes involved in sex determination in other cellular and organ contexts
  • CELLULAR PROCESS nucleotide, transcription, regulation
    metabolism lipid/lipoprotein
    signaling signal transduction
    a component
    DNA binding to DNA site A
    small molecule metal binding,
  • Zn2+
  • protein
  • functions as a transcriptional repressor of pro-testis genes
  • directly regulates the expression of both EYA1 and WT1 in the metanephric mesenchyme
  • interacts with SMAD4 to modulate TGFB1 signalling
  • FGF2 is an extracellular inducer of NR2F2 expression and may suppress the osteogenic potential of mesenchymal cells by inducing NR2F2 expression prior to the onset of osteogenic differentiation
  • cell & other
    corresponding disease(s) DEL15QD , DIH1 , VCHD
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    significantly impaired adult neoangiogenesis (Qin 2010)
    tumoral       gain of function
    upregulated in metastatic prostate cancer, and its higher expression correlated with a worse clinical outcome
    Susceptibility to nonsyndromic atrioventricular septal defects (AVSDs)
    Variant & Polymorphism other
  • variants in NR2F2 probably contribute to AVSDs and other CHDs during human development
  • Candidate gene for heart defects in del 15q patients
    for isolated congenital diaphragmatic hernia
    Therapy target
    inhibition of NR2F2 may offer a therapeutic approach for anticancer intervention
    . inhibition of NR2F2 suppresses metastases of mammary tumors to the lung
    is a potential drug target for the intervention of metastatic human prostate cancer
  • decreased dosage of Nr2f2 in mice has been shown to result in abnormal development of atrioventricular septa
  • mice lacking Coup-tf2 die around E10 due to defects of angiogenesis and heart development