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

More general terms

• hydrolase

Additional terms

• dihydropyrimidinase related protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=1807
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:1807
• OMIM: https://mirror.omim.org/entry/222748
• OMIM: https://mirror.omim.org/entry/613326
• UniProt: http://www.uniprot.org/uniprot/Q14117.html