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dihydropyrimidinase; DHP; DHPase; dihydropyrimidine amidohydrolase; hydantoinase (DPYS)
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 & of 5,6-dihydrothymine to N-carbamyl-amino isobutyrate
- carbamylation allows a single lysine to coordinate two Zn+2 ions (putative)
5,6-dihydrouracil + H2O = 3-ureidopropanoate
Cofactor: binds 2 Zn+2 per subunit
Structure:
- homotetramer (probable)
- belongs to the DHOase family hydantoinase/dihydropyrimidinase subfamily
Expression: liver & kidney
Pathology:
- defects in DPYS are the cause of dihydropyrimidinase deficiency
Related
dihydropyrimidinase related protein
General
hydrolase
Properties
SIZE: entity length = 519 aa
MW = 57 kD
MOTIF: Zn+2-binding site
SITE: 67-67
Zn+2-binding site
SITE: 69-69
Zn+2-binding site
SITE: 159-159
binding site
SITE: 164-164
FOR-BINDING-OF: Substrate
Zn+2-binding site
SITE: 192-192
Zn+2-binding site
SITE: 248-248
active site
Zn+2-binding site
SITE: 326-326
binding site
SITE: 347-347
FOR-BINDING-OF: Substrate; via carbonyl oxygen
Database Correlations
OMIM correlations
MORBIDMAP 613326
UniProt Q14117
Pfam PF01979
Entrez Gene 1807
KEGG correlations
ENZYME 3.5.2.2
References
- UniProt :accession Q14117
- GeneReviews
http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/DPYS