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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

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_003124 3 - 1432 NP_003115 - 261 - 2014 24096079

EXPRESSION

Type

widely

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol Digestive salivary gland       highly Endocrine parathyroid       highly Nervous brain       predominantly Skin/Tegument skin       highly

tissue

System Tissue Tissue level 1 Tissue level 2 Level Pubmed Species Stage Rna symbol Connective bone     highly

cells

System Cell Pubmed Species Stage Rna symbol Nervous neuron

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)

Type Gene Modification Chromosome rearrangement Protein expression Protein 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
	System Type Disorder Pubmed miscelleaneous pain   tranilast can act as a potent SPR inhibitor and therefore is a valid candidate for drug repurposing in the treatment of chronic pain immunology autoimmune articular SPR inhibition reduces pain associated with joint inflammation, showing potential utility as an analgesic strategy for inflammatory joint pain

ANIMAL & CELL MODELS