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Details on Person UniProt:P35270 SPR

Class:IdReferenceGeneProduct:64988
_chainChangeLogchain:1-261 added on Sat February 7 2015
_displayNameUniProt:P35270 SPR
_timestamp2025-08-15 21:57:12
chainchain:1-261
checksum9C9BF76212826F47
commentFUNCTION Catalyzes the final one or two reductions in tetra-hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin.CATALYTIC ACTIVITY L-erythro-7,8-dihydrobiopterin + NADP(+) = L-sepiapterin + NADPH + H(+)CATALYTIC ACTIVITY (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + 2 NADP(+) = 6-pyruvoyl-5,6,7,8-tetrahydropterin + 2 NADPH + 2 H(+)BIOPHYSICOCHEMICAL PROPERTIES kcat is 1.1 sec(-1) with sepiapterin as substrate (PubMed:10350607). kcat is 1.1 sec(-1) with NADPH as substrate (PubMed:10350607).SUBUNIT Homodimer.SUBCELLULAR LOCATION In vitro phosphorylation of Ser-213 by CaMK2 does not change kinetic parameters.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the sepiapterin reductase family.
descriptionrecommendedName: Sepiapterin reductase shortName: SPR ecNumber evidence="3 4"1.1.1.153
geneNameSPR
identifierP35270
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Cytoplasm
Disease variant
Dystonia
NADP
Oxidoreductase
Phosphoprotein
Proteomics identification
Reference proteome
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
nameSPR
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8997455] ENSEMBL:ENSG00000116096 SPR [Homo sapiens]
secondaryIdentifierSPRE_HUMAN
A8K741
D6W5H2
Q53GI9
Q9UBB1
sequenceLength261
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:1497782] p-S213-SPR [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:1497798] SPR [cytosol] [Homo sapiens]
(referenceSequence)[ModifiedResidue:1497776] O-phospho-L-serine at 213
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No pathways have been reviewed or authored by UniProt:P35270 SPR (64988)