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Symbol ENTPD1 contributors: mct - updated : 05-12-2016
HGNC name ectonucleoside triphosphate diphosphohydrolase 1
HGNC id 3363
Corresponding disease
SPG64 spastic paraplegia 64
Location 10q24.1      Physical location : 97.471.535 - 97.637.022
Synonym name
  • vascular ATP diphosphohydrolase
  • CD39 antigen
  • nucleoside triphosphate diphosphohydrolase (NTPDase)-1
  • cell differentiation antigen CD39, identified by monoclonal antibody B721
  • ecto-ATP diphosphohydrolase
  • ecto-apyrase
  • NTPDase 1
  • Synonym symbol(s) CD39, ATPDase, FLJ40921, FLJ40959, NTPDase-1, DKFZp686D194, DKFZp686I093, SPG64
    TYPE functioning gene
    SPECIAL FEATURE head to head
    text vascular ATP diphosphohydrolase
    STRUCTURE 165.49 kb     10 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    Map see WNT8B
    Physical map
    PLCE1 10q23 phospholipase C, epsilon 1 AD24 10q23.33 AD24 protein KIAA0608 10q23.33 KIAA0608 protein HELLS 10q23-q24 helicase, lymphoid-specific CYP2C18 10q24 cytochrome P450, family 2, subfamily C, polypeptide 18 CYP2C19 10q24 cytochrome P450, family 2, subfamily C, polypeptide 19 CYP2C9 10q24.1 cytochrome P450, family 2, subfamily C, polypeptide 9 CYP2C8 10q23.33 cytochrome P450, family 2, subfamily C, polypeptide 8 LOC387705 10 LOC387705 LOC142827 10q24.1 similar to Hypothetical protein MGC56918 PDLIM1 10q22-q26.3 PDZ and LIM domain 1 (elfin) SORBS1 10q23.3-q24.1 sorbin and SH3 domain containing 1 LOC389999 10 similar to ribosomal protein S3a; 40S ribosomal protein S3a; v-fos transformation effector protein 1 PYCS 10q24.3 pyrroline-5-carboxylate synthetase (glutamate gamma-semialdehyde synthetase) DKFZP564D116 10q24.1 DKFZP564D116 protein ENTPD1 10q24 ectonucleoside triphosphate diphosphohydrolase 1 LOC387706 10 LOC387706 LOC387707 10 similar to RIKEN cDNA 5730509K17 gene FLJ10895 10pter-q26.12 hypothetical protein FLJ10895 KIAA0335 10cen-q26.11 hypothetical protein FLJ10895 LOC387708 10 similar to nucleophosmin 1; nucleolar phosphoprotein B23; numatrin; nucleophosmin/nucleoplasmin family, member 1 BLNK 10q23.2 B-cell linker DNTT 10q24 deoxynucleotidyltransferase, terminal TMEM10 10q23-q24 transmembrane protein 10 TLL2 10q23-q24 tolloid-like 2 SMBP 10q24.2 SM-11044 binding protein PIK3AP1 10q24.2 phosphoinositide-3-kinase adaptor protein 1 LOC283340 10q24.2 similar to ribosomal protein L13a; 60S ribosomal protein L13a; 23 kD highly basic protein LOC387709 10 LOC387709 C10orf12 10q24.2 chromosome 10 open reading frame 12 SLIT1 10q23.3-q24 slit homolog 1 (Drosophila) ARHGAP19 10q24.2 Rho GTPase activating protein 19 FRAT1 10q24.1 frequently rearranged in advanced T-cell lymphomas FRAT2 10q23-q24.1 frequently rearranged in advanced T-cell lymphomas 2 KIAA0690 10q23.33 frequently rearranged in advanced T-cell lymphomas 2 PGAM1 10q25.3 phosphoglycerate mutase 1 (brain) CSL4 10q24 exosomal core protein CSL4 ZDHHC16 10q24.1 zinc finger, DHHC domain containing 16 MMS19L 10q24-q25 MMS19-like (MET18 homolog, S. cerevisiae) FLJ11807 10q24.2 hypothetical protein FLJ11807
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    10 - 12887 - 510 - 2015 26226423
    9 - 12615 - 517 - 2015 26226423
    10 - 12515 - 522 - 2015 26226423
    10 - 12617 - 469 - 2015 26226423
    9 - 12387 - 402 - 2015 26226423
    9 - 12589 - 372 - 2015 26226423
    8 - 12471 - 372 - 2015 26226423
    10 - 12561 - 402 - 2015 26226423
    10 - 2171 - 482 - 2015 26226423
    Type ubiquitous
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Endocrineparathyroid   highly
    Hearing/Equilibriumear   highly Homo sapiens
    Reproductivefemale systemplacenta  highly Homo sapiens
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    SystemCellPubmedSpeciesStageRna symbol
    cell lineage
    cell lines
    at STAGE
    physiological period pregnancy
    Text placenta
  • basic leucine zipper (bZIP) like structure
  • four apyrase conserved regions in N terminal region (ACR)
  • site of binding to RANBP9
  • conjugated GlycoP
    interspecies homolog to rattus brain E type ecto ATPase
  • GDA1/CD39 NTPase family
  • ectonucleoside triphosphate diphosphohydrolase (ENTPDase) family
  • CATEGORY adhesion , enzyme , transcription factor , antigen
    SUBCELLULAR LOCALIZATION     plasma membrane
  • localized in caveolae, which are plasma membrane invaginations with distinct lipid composition, similar to dynamic lipid microdomains, called rafts
  • expressed at the sympathetic neuromuscular junction, where ATP is co-released with norepinephrine
  • basic FUNCTION
  • ectoenzyme that degrades ATP to AMP
  • modulating P2 receptor signaling by controlling nucleotides concentrations at the cell surface
  • ecto-nucleotidase influencing P2 receptor activation to regulate vascular and immune cell adhesion and signaling events pivotal in inflammation
  • regulates purinergic receptor signaling by controlling the levels of extracellular nucleotides
  • having a role in thromboregulation (through its localization in cholesterol-rich domains of the membrane)
  • by hydrolyzing ATP and ADP to AMP, regulates ligand availability to a large family of P2 (purinergic) receptors (Friedman 2007)
  • vascular protective factor in diabetic nephropathy that modulates glomerular inflammation and thromboregulation (Friedman 2007)
  • controls IL8 production by human neutrophils via the regulation of P2Y(2) activation
  • has anti-inflammatory properties as it hydrolyzes proinflammatory extracellular ATP, generates anti-inflammatory adenosine, and also protects regulatory T cells from ATP-induced cell death
  • is an integral component of regulatory T cells (Treg), which are central to immunological tolerance and maintenance of normal pregnancy
  • cellular homeostasis and fibrotic response involve the integration of signaling that is pro-fibrotic by ATP and anti-fibrotic by adenosine and that is regulated by ENTPD1 and ENTPD2
  • is a key "molecular switch" that allows macrophages to self-limit their activation state
  • extracellular adenosine, generated in tandem by ecto-enzymes ENTPD1 and NT5E, promotes dermal fibrogenesis
  • ENTPD1 modulates local renin release and thus, RAS activation, ultimately exerting a cardioprotective effect
  • expression of ENTPD1 in Tregs is primarily genetically driven, and this may determine interindividual differences in the control of inflammatory responses
  • marker of regulatory immune cells and catalyzes extracellular hydrolysis of nucleotides to generate AMP and, in tandem with NT5E, adenosine
  • ENTPD1, NT5E are two ectonucleotidases that cooperate in the generation of extracellular adenosine through ATP hydrolysis, thus tilting the balance towards immunosuppressive microenvironments
  • catalyzes the phosphohydrolysis of extracellular ATP (eATP) and ADP (eADP) released under conditions of inflammatory stress and cell injury
  • plays a dominant role in the purinergic regulation of inflammation and the immune response because its expression is influenced by genetic and environmental factors
  • CELLULAR PROCESS cell life, cell death/apoptosis
    nucleotide, transcription
    PHYSIOLOGICAL PROCESS coagulation/hemostasis , immunity/defense
    text prevention of platelet aggregation
    a component
    small molecule
  • associations with RANBP9 having the potential to regulate NTPDase catalytic activity( this intermolecular interaction may have important implications for the regulation of extracellular nucleotide-mediated signaling)
  • is a negative regulator of P2RX7-mediated inflammatory cell death in mast cells
  • KLRB1 and ENTPD1 have direct interactions that are further linked with acid sphingomyelinase (ASM)
  • cell & other
    corresponding disease(s) SPG64
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    correlated with better long-term survival after tumor resection in patients with pancreatic cancer
    tumoral     --over  
    in malignant epithelial cells of human rectal adenocarcinoma
    Variant & Polymorphism
    Candidate gene marker of a Treg subset likely involved in the control of the inflammatory autoimmune disease
    Therapy target
    biochemical or biological inhibitors of ENTPD1 and/or NT5E may hold promise in the treatment of dermal fibrosis in diseases such as scleroderma
    may serve as a future target for the development of novel therapies with immune-modulating antitumor agents in chronic lymphocytic leukemia
  • Entpd1 null mice exhibit decreased synaptic efficacy, presumably due to desensitization P2X1 receptors
  • in rat models of DNA damage, inhibiting Entpd1-driven ATP hydrolysis with POM-1 protected the heart and lung tissues against chemically induced DNA damage