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FLASH GENE
Symbol SPR contributors: mct - updated : 12-11-2019
HGNC name sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase)
HGNC id 11257
EXPRESSION
Type widely
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestivesalivary gland   highly
Endocrineparathyroid   highly
Nervousbrain   predominantly
Skin/Tegumentskin   highly
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Connectivebone  highly
cells
SystemCellPubmedSpeciesStageRna symbol
Nervousneuron
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
mono polymer homomer , dimer
HOMOLOGY
interspecies homolog to murine Spr
Homologene
FAMILY aldoketoreductase family
CATEGORY enzyme
SUBCELLULAR LOCALIZATION     intracellular
intracellular,cytoplasm,cytosolic
intracellular,nucleus,nucleoplasm
basic FUNCTION
  • catalyzing the final step of tetrahydrobiopterin (BH4) synthesis, cofactor for aromatic aminoacid hydroxylase and nitric oxid synthase
  • important role of endothelial SPR in modulating tetrahydrobiopterin (H(4)B) and nitric oxide (NO(*) bioavailability
  • SPR is a key enzyme mediating chemical redox cycling suggests that it may be important in generating cytotoxic reactive oxygen species in the lung
  • mediates chemical redox cycling in lung epithelial cells
  • mediates chemical redox cycling, catalyzing one-electron reduction of redox-active chemicals, including quinones
  • GCH1, SPR and their downstream metabolite BH4 are critical regulators of T cell biology that can be readily manipulated to either block autoimmunity or enhance anticancer immunity
  • catalyzes both the reversible reduction of sepiapterin to dihydrobiopterin (BH2) and 6-pyruvoyl-tetrahydrobiopterin to BH4 within the BH4 pathway
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • SPR is a novel regulator of ODC1 enzyme activity and, potentially SPR drives ODC1-mediated malignant progression in neuroblastoma
  • cell & other
    REGULATION
    inhibited by xanthurenic acid that is a potent inhibitor of SPR, the final enzyme in de novo BH4 synthesis
    ASSOCIATED DISORDERS
    corresponding disease(s) SPRD
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    is significantly correlated to unfavorable Neuroblastoma (NB) characteristics like high age at diagnosis
    Susceptibility
    Variant & Polymorphism
    Candidate gene for PARK3
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    miscelleaneouspain 
    tranilast can act as a potent SPR inhibitor and therefore is a valid candidate for drug repurposing in the treatment of chronic pain
    immunologyautoimmunearticular
    SPR inhibition reduces pain associated with joint inflammation, showing potential utility as an analgesic strategy for inflammatory joint pain
    ANIMAL & CELL MODELS