Homo sapiens Protein: DPYS
Summary
InnateDB Protein IDBP-32050.4
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol DPYS
Protein Name dihydropyrimidinase
Synonyms DHP; DHPase;
Species Homo sapiens
Ensembl Protein ENSP00000276651
InnateDB Gene IDBG-32048 (DPYS)
Protein Structure
UniProt Annotation
Function Catalyzes the second step of the reductive pyrimidine degradation, the reversible hydrolytic ring opening of dihydropyrimidines. Can catalyze the ring opening of 5,6- dihydrouracil to N-carbamyl-alanine and of 5,6-dihydrothymine to N-carbamyl-amino isobutyrate.
Subcellular Localization
Disease Associations Dihydropyrimidinase deficiency (DHPD) [MIM:222748]: A disorder characterized by dihydropyrimidinuria and associated with a variable clinical phenotype characterized by epileptic or convulsive attacks, dysmorphic features and severe developmental delay, and congenital microvillous atrophy. {ECO:0000269PubMed:9718352}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity Liver and kidney.
Comments
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 0 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.
Predicted by orthology
Total 1 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0002058 uracil binding
GO:0002059 thymine binding
GO:0004157 dihydropyrimidinase activity
GO:0008270 zinc ion binding
GO:0016597 amino acid binding
GO:0016787 hydrolase activity
GO:0016810 hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds
GO:0016812 hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in cyclic amides
GO:0051219 phosphoprotein binding
Biological Process
GO:0006206 pyrimidine nucleobase metabolic process
GO:0006208 pyrimidine nucleobase catabolic process
GO:0006210 thymine catabolic process
GO:0006212 uracil catabolic process
GO:0019482 beta-alanine metabolic process
GO:0019860 uracil metabolic process
GO:0044281 small molecule metabolic process
GO:0046135 pyrimidine nucleoside catabolic process
GO:0051260 protein homooligomerization
GO:0051289 protein homotetramerization
GO:0055086 nucleobase-containing small molecule metabolic process
Cellular Component
GO:0005737 cytoplasm
GO:0005829 cytosol
GO:0070062 extracellular vesicular exosome
Protein Structure and Domains
PDB ID
InterPro IPR006680 Amidohydrolase 1
IPR011059 Metal-dependent hydrolase, composite domain
IPR011778 Hydantoinase/dihydropyrimidinase
PFAM PF01979
PRINTS
PIRSF
SMART
TIGRFAMs
Post-translational Modifications
Modification
Cross-References
SwissProt Q14117
PhosphoSite PhosphoSite-Q14117
TrEMBL
UniProt Splice Variant
Entrez Gene 1807
UniGene Hs.713087
RefSeq NP_001376
HUGO HGNC:3013
OMIM 613326
CCDS CCDS6302
HPRD 01960
IMGT
EMBL AB004678 BC034395 D78011
GenPept AAH34395 BAA11189 BAA33067