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Identification
HMDB Protein ID HMDBP00543
Secondary Accession Numbers
  • 5814
Name Dihydropyrimidinase
Synonyms
  1. DHP
  2. DHPase
  3. Dihydropyrimidine amidohydrolase
  4. Hydantoinase
Gene Name DPYS
Protein Type Unknown
Biological Properties
General Function Involved in hydrolase activity
Specific 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.
Pathways
  • Beta Ureidopropionase Deficiency
  • Beta-Alanine Metabolism
  • beta-Alanine metabolism
  • Carnosinuria, carnosinemia
  • Dihydropyrimidinase Deficiency
  • Drug metabolism - other enzymes
  • GABA-Transaminase Deficiency
  • MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy)
  • Pantothenate and CoA biosynthesis
  • Pyrimidine Metabolism
  • Pyrimidine metabolism
  • UMP Synthase Deficiency (Orotic Aciduria)
  • Ureidopropionase Deficiency
Reactions
Dihydrouracil + Water → Ureidopropionic acid details
Dihydrothymine + Water → Ureidoisobutyric acid details
5,6-Dihydro-5-fluorouracil + Water → alpha-Fluoro-beta-ureidopropionic acid details
GO Classification
Biological Process
pyrimidine nucleoside catabolic process
thymine catabolic process
uracil catabolic process
beta-alanine metabolic process
protein homotetramerization
Cellular Component
cytosol
Function
catalytic activity
hydrolase activity
hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds
Molecular Function
metal ion binding
zinc ion binding
dihydropyrimidinase activity
thymine binding
uracil binding
amino acid binding
Cellular Location
  1. Cytoplasmic
Gene Properties
Chromosome Location 8
Locus 8q22
SNPs DPYS
Gene Sequence
>1560 bp
ATGGCGGCGCCCTCGCGGCTCCTGATCCGCGGGGGTCGCGTGGTCAACGATGACTTCTCG
GAGGTGGCCGACGTGCTGGTGGAGGACGGCGTGGTGCGGGCACTCGGGCACGACCTGCTG
CCTCCCGGGGGCGCTCCTGCGGGGCTGCGGGTCCTCGACGCCGCCGGCAAGCTCGTCCTG
CCCGGAGGCATCGACACACACACGCACATGCAGTTCCCCTTCATGGGCTCGCGGTCCATC
GACGACTTCCACCAGGGCACCAAGGCTGCTCTCTCAGGAGGCACCACCATGATTATTGAT
TTCGCCATTCCTCAGAAAGGTGGCTCCCTCATTGAGGCCTTCGAGACCTGGCGAAGCTGG
GCTGATCCCAAAGTTTGCTGCGACTACAGCCTTCATGTGGCAGTGACGTGGTGGAGTGAC
CAGGTTAAAGAAGAAATGAAAATCCTTGTGCAAGATAAAGGTGTTAACTCTTTCAAGATG
TTTATGGCCTATAAAGATCTGTACATGGTGACAGACCTGGAGCTGTACGAAGCCTTCTCT
CGGTGCAAGGAAATTGGAGCAATTGCCCAGGTCCATGCGGAAAATGGAGACTTAATTGCA
GAGGGAGCAAAGAAGATGTTGGCTCTGGGGATAACAGGCCCTGAGGGCCACGAGCTGTGC
CGCCCAGAGGCAGTGGAGGCAGAGGCCACGCTGAGAGCCATCACCATAGCCAGCGCTGTG
AACTGTCCTCTCTACATTGTGCATGTGATGAGCAAGTCTGCAGCTAAGGTGATAGCGGAT
GCAAGGAGAGATGGGAAGGTGGTCTATGGTGAACCCATAGCAGCCAGTCTTGGCACAGAT
GGCACTCACTACTGGAATAAAGAATGGCACCATGCAGCCCACCATGTCATGGGTCCACCT
TTGCGACCAGACCCCTCAACACCCGACTTCCTCATGAATCTGTTGGCTAATGATGATCTA
ACCACAACAGGGACTGATAACTGCACTTTCAACACCTGCCAGAAAGCTCTTGGGAAGGAT
GATTTTACCAAGATCCCCAATGGGGTGAATGGTGTTGAAGATCGGATGTCCGTAATATGG
GAAAAAGGCGTGCATAGTGGTAAAATGGATGAAAACAGATTTGTGGCAGTTACCAGCACA
AATGCAGCCAAAATTTTTAATCTCTATCCAAGAAAAGGAAGAATAGCTGTAGGATCAGAT
GCTGACATTGTTATTTGGGACCCAAAAGGCACAAGGACTATCTCAGCAAAAACTCATCAT
CAGGCTGTTAACTTCAACATTTTCGAGGGCATGGTTTGCCACGGGGTGCCCCTTGTGACT
ATTTCAAGAGGCAAAGTGGTATATGAAGCCGGAGTGTTCAGTGTCACGGCAGGAGATGGG
AAGTTTATTCCTCGAAAACCATTTGCTGAATATATTTACAAACGAATAAAGCAGCGAGAC
CGGACTTGCACACCTACCCCTGTGGAGCGTGCACCCTATAAGGGAGAAGTCGCCACACTG
AAATCCAGAGTGACAAAAGAAGATGCCACAGCAGGGACCAGGAAACAGGCCCACCCCTGA
Protein Properties
Number of Residues 519
Molecular Weight 56629.36
Theoretical pI 7.263
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Dihydropyrimidinase
MAAPSRLLIRGGRVVNDDFSEVADVLVEDGVVRALGHDLLPPGGAPAGLRVLDAAGKLVL
PGGIDTHTHMQFPFMGSRSIDDFHQGTKAALSGGTTMIIDFAIPQKGGSLIEAFETWRSW
ADPKVCCDYSLHVAVTWWSDQVKEEMKILVQDKGVNSFKMFMAYKDLYMVTDLELYEAFS
RCKEIGAIAQVHAENGDLIAEGAKKMLALGITGPEGHELCRPEAVEAEATLRAITIASAV
NCPLYIVHVMSKSAAKVIADARRDGKVVYGEPIAASLGTDGTHYWNKEWHHAAHHVMGPP
LRPDPSTPDFLMNLLANDDLTTTGTDNCTFNTCQKALGKDDFTKIPNGVNGVEDRMSVIW
EKGVHSGKMDENRFVAVTSTNAAKIFNLYPRKGRIAVGSDADIVIWDPKGTRTISAKTHH
QAVNFNIFEGMVCHGVPLVTISRGKVVYEAGVFSVTAGDGKFIPRKPFAEYIYKRIKQRD
RTCTPTPVERAPYKGEVATLKSRVTKEDATAGTRKQAHP
GenBank ID Protein 3608122
UniProtKB/Swiss-Prot ID Q14117
UniProtKB/Swiss-Prot Entry Name DPYS_HUMAN
PDB IDs
GenBank Gene ID AB004678
GeneCard ID DPYS
GenAtlas ID DPYS
HGNC ID HGNC:3013
References
General References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  2. Hamajima N, Matsuda K, Sakata S, Tamaki N, Sasaki M, Nonaka M: A novel gene family defined by human dihydropyrimidinase and three related proteins with differential tissue distribution. Gene. 1996 Nov 21;180(1-2):157-63. [PubMed:8973361 ]
  3. Hamajima N, Kouwaki M, Vreken P, Matsuda K, Sumi S, Imaeda M, Ohba S, Kidouchi K, Nonaka M, Sasaki M, Tamaki N, Endo Y, De Abreu R, Rotteveel J, van Kuilenburg A, van Gennip A, Togari H, Wada Y: Dihydropyrimidinase deficiency: structural organization, chromosomal localization, and mutation analysis of the human dihydropyrimidinase gene. Am J Hum Genet. 1998 Sep;63(3):717-26. [PubMed:9718352 ]