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Human Metabolome Database Version 2.5

 

Showing metabocard for Orotic acid (HMDB00226)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2005-11-16 15:48:42
Update Date 2010-03-16 13:35:11
Accession Number HMDB00226
Secondary Accession Numbers Not Available
Common Name Orotic acid
Description Orotic acid is a minor dietary constituent. Indeed, until it was realized that it could be synthesized by humans, orotic acid was known as vitamin B-13. The richest dietary sources are cow's milk and other dairy products as well as root vegetables such as carrots and beets. Dietary intake probably contributes to a basal rate of orotic acid excretion in urine because fasting decreases excretion by ~50%. However, it is now apparent that most urinary orotic acid is synthesized in the body, where it arises as an intermediate in the pathway for the synthesis of pyrimidine nucleotides. Orotic acid is converted to UMP by UMP synthase, a multifunctional protein with both orotate phosphoribosyltransferase and orotidylate decarboxylase activity. The most frequently observed inborn error of pyrimidine nucleotide synthesis is a mutation of the multifunctional protein UMP synthase. This disorder prevents the conversion of orotic acid to UMP and thus to other pyrimidines. As a result, plasma orotic acid accumulates to high concentrations, and increased quantities appear in the urine. Indeed, urinary orotic acid is so markedly increased in individuals harboring a mutation in UMP synthase that orotic acid crystals can form in the urine. The urinary concentration of orotic acid in homozygotes can be of the order of millimoles per millimole creatinine. By comparison, the urinary level in unaffected individuals is ~ 1 umol/mmol creatinine. (PMID: 17513443)
Synonyms
  1. uracil-6-carboxylate
  2. acidum oroticum
  3. Whey factor
  4. Vitamin B13
  5. Uracil-6-carboxylic acid
  6. Uracil-6-carbosaeure
  7. Orotyl
  8. Oroturic
  9. Orotsaure
  10. Orotsaeure
  11. Orotonsan
  12. Orotonin
  13. Orotic acid
  14. Orotate
  15. Oropur
  16. Orodin
  17. ORO
  18. Molkensaeure
  19. Lactinium
  20. Animal galactose factor
  21. acido orotico
  22. acide orotique
  23. 6-Uracilcarboxylic acid
  24. 6-Carboxyuracil
  25. 6-Carboxy-2,4-dihydroxypyrimidine
  26. 2,6-dioxo-1,2,3,6-tetrahydropyrimidine-4-carboxylic acid
  27. 2,6-dihydroxy-4-pyrimidinecarboxylic acid
  28. 2,6-Dioxo-1,2,3,6-tetrahydro-pyrimidine-4-carboxylic acid
  29. 2,6-Dihydroxypyrimidine-4-carboxylic acid
  30. 1,2,3,6-tetrahydro-2,6-dioxo-4-Pyrimidinecarboxylic acid
  31. 1,2,3,6-Tetrahydro-2,6-dioxopyrimidin-4-carbonsaeure
  32. 1,2,3,6-Tetrahydro-2,6-dioxo-4-pyrimidecarboxylic acid
Chemical IUPAC Name 2,6-dioxo-3H-pyrimidine-4-carboxylic acid
Chemical Formula C5H4N2O4
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Organic acids
Class
  • Pyrimidines and Derivatives
Sub Class
  • Aromatic keto-acids
Family
  • Mammalian Metabolite
Species
  • carboxylic acid
  • oxo(het)arene
  • aromatic compound
  • heterocyclic compound
Biofunction
  • Component of Pyrimidine metabolism
Application
—
Source
  • Endogenous
Average Molecular Weight 156.096
Monoisotopic Molecular Weight 156.017105
Isomeric SMILES OC(=O)C1=CC(=O)NC(=O)N1
Canonical SMILES OC(=O)C1=CC(=O)NC(=O)N1
KEGG Compound ID C00295 Link Image
BioCyc ID OROTATE Link Image
BiGG ID 34527 Link Image
Wikipedia Link Orotic acid Link Image
NuGOwiki Link HMDB00226 Link Image
Metagene Link HMDB00226 Link Image
METLIN ID 318 Link Image
PubChem Compound 967 Link Image
PubChem Substance 3589 Link Image
ChEBI ID 16742 Link Image
CAS Registry Number 65-86-1
InChI Identifier InChI=1/C5H4N2O4/c8-3-1-2(4(9)10)6-5(11)7-3/h1H,(H,9,10)(H2,6,7,8,11)
Synthesis Reference Mitchell, Herschel K.; Nyc, Joseph F. Intermediates in the synthesis of orotic acid from oxalacetic ester and urea. Journal of the American Chemical Society (1947), 69 674-7.
Melting Point (Experimental) 345.5 oC
Experimental Water Solubility 1.82 mg/mL at 18 oC [YALKOWSKY,SH & DANNENFELSER,RM (1992)] Source: PhysProp
Predicted Water Solubility 4.51 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -1
State Solid
Experimental LogP/Hydrophobicity -0.83 [SANGSTER (1994)] Source: PhysProp
Predicted LogP/Hydrophobicity -0.89 [Predicted by ALOGPS]; -3.4 [Predicted by PubChem via XLOGP] Calculated using ALOGPS
Material Safety Data Sheet (MSDS)
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID 1D3G Link Image
Experimental PDB File Show
Experimental PDB Structure
Experimental 1H NMR Spectrum Download Spectrum
Download FID (Varian)
Show Experimental Conditions Link Image
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Download Spectrum
Download FID (Bruker)
Show Experimental Conditions Link Image
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum
Low Energy
Download File
Show Experimental Conditions Link Image
Medium Energy
Download File
Show Experimental Conditions Link Image
High Energy
Download File
Show Experimental Conditions Link Image
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Show Image
Show Peaklist
Cellular Location
  • Cytoplasm (Predicted from LogP)
Biofluid Location
  • Amniotic Fluid
  • Blood
  • Urine
Tissue Location
Tissue References
Liver —
Pancreas —
Concentrations (Normal)
Biofluid Amniotic Fluid
Value 0.27 +/- 0.25 uM
Age Adult:>18 yrs old
Sex Female
Patient information Normal
Comments Not Available
References
  • Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed Link Image]
Biofluid Blood
Value 5.0 +/- 0.4 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Traut TW: Physiological concentrations of purines and pyrimidines. Mol Cell Biochem. 1994 Nov 9;140(1):1-22. [PubMed Link Image]
Biofluid Blood
Value 0.89 +/- 0.63 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed Link Image]
Biofluid Urine
Value 1.05 +/- 1.22 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Paradis D, Giguere R, Auray-Blais C, Draper P, Lemieux B: An automated method for the determination of orotic acid in the urine of children being screened for metabolic disorders. Clin Biochem. 1980 Aug;13(4):160-3. [PubMed Link Image]
Biofluid Urine
Value 0.0013+/-0.0008 umol/mmol creatinine
Age N/A
Sex Both
Patient information Normal
Comments Not Available
References
  • Arranz JA, Riudor E, Rodes M, Roig M, Climent C, Rubio V, Sentis M, Burlina A: Optimization of allopurinol challenge: sample purification, protein intake control, and the use of orotidine response as a discriminative variable improve performance of the test for diagnosing ornithine carbamoyltransferase deficiency. Clin Chem. 1999 Jul;45(7):995-1001. [PubMed Link Image]
Biofluid Urine
Value 0.0011+/-0.0008 umol/mmol creatinine
Age N/A
Sex Both
Patient information Normal
Comments Not Available
References
  • Paradis D, Giguere R, Auray-Blais C, Draper P, Lemieux B: An automated method for the determination of orotic acid in the urine of children being screened for metabolic disorders. Clin Biochem. 1980 Aug;13(4):160-3. [PubMed Link Image]
Biofluid Urine
Value 0.23 +/- 0.13 umol/mmol creatinine
Age Children:1-13 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed Link Image]
Biofluid Urine
Value 0.16 +/- 0.12 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed Link Image]
Biofluid Urine
Value 1.05 +/- 1.22 umol/mmol creatinine
Age Infant:0-1 yr old
Sex Both
Patient information Normal
Comments Not Available
References
  • Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed Link Image]
Biofluid Urine
Value 0.63 +/- 0.21 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Biofluid Urine
Value 5.5 (0.00-11.00) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
Concentrations (Abnormal)
Biofluid Blood
Value 0.94 +/- 0.78 uM
Age Adult:>18 yrs old
Sex Both
Condition Canavan disease
Comments Not Available
References
  • Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed Link Image]
Biofluid Urine
Value 6.11 +/- 5.48 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Condition Canavan disease
Comments Not Available
References
  • Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed Link Image]
Biofluid Urine
Value 2.5 (0.00-5.00) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Comments Not Available
References
Biofluid Urine
Value 3500.00 (1400.00-5600.00) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Comments Not Available
References
Associated Disorders
Condition References
Canavan disease
  • Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed Link Image]
OMIM ID
Pathways
Name SMPDB Link KEGG Link
Pyrimidine Metabolism SMP00046 Link Image map00240 Link Image
General References
  1. Zieve L: Conditional deficiencies of ornithine or arginine. J Am Coll Nutr. 1986;5(2):167-76. [PubMed Link Image]
  2. Visek WJ: Ammonia: its effects on biological systems, metabolic hormones, and reproduction. J Dairy Sci. 1984 Mar;67(3):481-98. [PubMed Link Image]
  3. Arranz JA, Riudor E, Rodes M, Roig M, Climent C, Rubio V, Sentis M, Burlina A: Optimization of allopurinol challenge: sample purification, protein intake control, and the use of orotidine response as a discriminative variable improve performance of the test for diagnosing ornithine carbamoyltransferase deficiency. Clin Chem. 1999 Jul;45(7):995-1001. [PubMed Link Image]
  4. Sugio K, Gazdar AF, Albores-Saavedra J, Kokkinakis DM: High yields of K-ras mutations in intraductal papillary mucinous tumors and invasive adenocarcinomas induced by N-nitroso(2-hydroxypropyl)(2-oxopropyl)amine in the pancreas of female Syrian hamsters. Carcinogenesis. 1996 Feb;17(2):303-9. [PubMed Link Image]
  5. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed Link Image]
  6. Finkelstein JE, Hauser ER, Leonard CO, Brusilow SW: Late-onset ornithine transcarbamylase deficiency in male patients. J Pediatr. 1990 Dec;117(6):897-902. [PubMed Link Image]
  7. Harris ML, Oberholzer VG: Conditions affecting the colorimetry of orotic acid and orotidine in urine. Clin Chem. 1980 Mar;26(3):473-9. [PubMed Link Image]
  8. Jakobs C, Sweetman L, Nyhan WL, Gruenke L, Craig JC, Wadman SK: Stable isotope dilution analysis of orotic acid and uracil in amniotic fluid. Clin Chim Acta. 1984 Nov 15;143(2):123-33. [PubMed Link Image]
  9. Visek WJ: Arginine and disease states. J Nutr. 1985 Apr;115(4):532-41. [PubMed Link Image]
  10. Mizutani Y, Wada H, Fukushima M, Yoshida O, Nakanishi H, Li YN, Miki T: Prognostic significance of orotate phosphoribosyltransferase activity in bladder carcinoma. Cancer. 2004 Feb 15;100(4):723-31. [PubMed Link Image]
  11. Valik D, Sedova Z, Starha J, Zeman J, Hruba E, Dvorakova L: Acute hyperammonaemic encephalopathy in a female newborn caused by a novel, de novo mutation in the ornithine transcarbamylase gene. Acta Paediatr. 2004 May;93(5):710-1. [PubMed Link Image]
  12. Mills GC, Schmalstieg FC, Newkirk KE, Goldblum RM: Cytosine and orotic acid in urine of immunodeficient children. Clin Chem. 1979 Mar;25(3):419-24. [PubMed Link Image]
  13. Paradis D, Giguere R, Auray-Blais C, Draper P, Lemieux B: An automated method for the determination of orotic acid in the urine of children being screened for metabolic disorders. Clin Biochem. 1980 Aug;13(4):160-3. [PubMed Link Image]
  14. Potter M, Hammond JW, Sim KG, Green AK, Wilcken B: Ornithine carbamoyltransferase deficiency: improved sensitivity of testing for protein tolerance in the diagnosis of heterozygotes. J Inherit Metab Dis. 2001 Feb;24(1):5-14. [PubMed Link Image]
  15. van Kuilenburg AB, van Lenthe H, Loffler M, van Gennip AH: Analysis of pyrimidine synthesis "de novo" intermediates in urine and dried urine filter- paper strips with HPLC-electrospray tandem mass spectrometry. Clin Chem. 2004 Nov;50(11):2117-24. Epub 2004 Sep 16. [PubMed Link Image]
  16. Wikipedia Link Image
Metabolic Enzymes
  1. Dihydroorotate dehydrogenase, mitochondrial precursor
  2. Uridine 5'-monophosphate synthase
  3. cDNA FLJ75687, highly similar to Homo sapiens uridine monophosphate synthetase
Enzyme 1 [top]
Enzyme 1 ID 5416
Enzyme 1 Name Dihydroorotate dehydrogenase, mitochondrial precursor
Enzyme 1 Synonyms
  1. Dihydroorotate oxidase
  2. DHOdehase
Enzyme 1 Gene Name DHODH
Enzyme 1 Protein Sequence >Dihydroorotate dehydrogenase, mitochondrial precursor 
MAWRHLKKRAQDAVIILGGGGLLFASYLMATGDERFYAEHLMPTLQGLLDPESAHRLAVR
FTSLGLLPRARFQDSDMLEVRVLGHKFRNPVGIAAGFDKHGEAVDGLYKMGFGFVEIGSV
TPKPQEGNPRPRVFRLPEDQAVINRYGFNSHGLSVVEHRLRARQQKQAKLTEDGLPLGVN
LGKNKTSVDAAEDYAEGVRVLGPLADYLVVNVSSPNTAGLRSLQGKAELRRLLTKVLQER
DGLRRVHRPAVLVKIAPDLTSQDKEDIASVVKELGIDGLIVTNTTVSRPAGLQGALRSET
GGLSGKPLRDLSTQTIREMYALTQGRVPIIGVGGVSSGQDALEKIRAGASLVQLYTALTF
WGPPVVGKVKRELEALLKEQGFGGVTDAIGADHRR
Enzyme 1 Number of Residues 395
Enzyme 1 Molecular Weight 42868
Enzyme 1 Theoretical pI 10.23
Enzyme 1 GO Classification
Function
  • catalytic activity
  • dihydroorotate dehydrogenase activity
  • dihydroorotate oxidase activity
  • oxidoreductase activity
  • oxidoreductase activity, acting on the CH-CH group of donors
  • oxidoreductase activity, acting on the CH-CH group of donors, oxygen as acceptor
Process
  • 'de novo' pyrimidine base biosynthesis
  • UMP biosynthesis
  • cellular metabolism
  • metabolism
  • nucleobase metabolism
  • nucleobase, nucleoside, nucleotide and nucleic acid metabolism
  • nucleotide metabolism
  • physiological process
  • pyrimidine base biosynthesis
  • pyrimidine base metabolism
  • pyrimidine nucleoside monophosphate biosynthesis
  • pyrimidine nucleotide biosynthesis
  • pyrimidine nucleotide metabolism
  • pyrimidine ribonucleoside monophosphate biosynthesis
Component
  • cell
  • membrane
Enzyme 1 General Function Nucleotide transport and metabolism
Enzyme 1 Specific Function (S)-dihydroorotate + O(2) = orotate + H(2)O(2)
Enzyme 1 Pathways
Enzyme 1 Reactions
  • (S)-dihydroorotate + O2 = orotate + H2O2
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • 1-25
Enzyme 1 Transmembrane Regions Not Available
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 555594 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID Q02127 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name PYRD_HUMAN Link Image
Enzyme 1 PDB ID 1D3H Link Image
Enzyme 1 PDB File Show
Enzyme 1 3D Structure
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >1191 bp 
AAATTACCGTGGAGACACCTGCAAAAGCGGGCCCAGGATGCTGTGATCATCCTGGGGGGA
GGAGGACTTCTCTTCGCCTCCTACCTGATGGCCACGGGAGATGAGCGTTTCTATGCTGAA
CACCTGATGCCGACTCTGCAGGGGCTGCTGGACCCGGAGTCAGCCCACAGACTGGCTGTT
CGCTTCACCTCCCTGGGGCTCCTTCCACGGGCCAGATTTCAAGACTCTGACATGCTGGAA
GTGAGAGTTCTGGGCCATAAATTCCGAAATCCAGTAGGAATTGCTGCAGGATTTGACAAG
CATGGGGAAGCCGTGGACGGACTTTATAAGATGGGCTTTGGTTTTGTTGAGATAGGAAGT
GTGACTCCAAAACCTCAGGAAGGAAACCCTAGACCCAGAGTCTTCCGCCTCCCTGAGGAC
CAAGCTGTCATTAACAGGTATGGATTTAACAGTCACGGGCTTTCAGTGGTGGAACACAGG
TTACGGGCCAGACAGCAGAAGCAGGCCAAGCTCACAGAAGATGGACTGCCTCTGGGGGTC
AACTTGGGGAAGAACAAGACCTCAGTGGACGCCGCGGAGGACTACGCAGAAGGGGTGCGC
GTACTGGGCCCCCTGGCCGACTACCTGGTGGTGAATGTGTCCAGCCCCAACACTGCCGGG
CTGCGGAGCCTTCAGGGAAAGGCCGAGCTGCGCCGCCTGCTGACCAAGGTGCTGCAGGAG
AGGGATGGCTTGCGGAGAGTGCACAGGCCGGCAGTCCTGGTGAAGATCGCTCCTGACCTC
ACCAGCCAGGATAAGGAGGACATTGCCAGTGTGGTCAAAGAGTTGGGCATCGATGGGCTG
ATTGTTACGAACACCACCGTGAGTCGCCCTGCGGGCCTCCAGGGTGCCCTGCGCTCTGAA
ACAGGAGGGCTGAGTGGGAAGCCCCTCCGGGATTTATCAACTCAAACCATTCGGGAGATG
TATGCACTCACCCAAGGCCGAGTTCCCATAATTGGGGTTGGTGGTGTGAGCAGCGGGCAG
GACGCGCTGGAGAAGATCCGGGCAGGGGCCTCCCTGGTGCAGCTGTACACGGCCCTCACC
TTCTGGGGGCCACCCGTTGTGGGCAAAGTCAAGCGGGAACTGGAGGCCCTTCTGAAAGAG
CAGGGCTTTGGCGGAGTCACAGATGCCATTGGAGCAGATCATCGGAGGTGA
Enzyme 1 GenBank Gene ID M94065 Link Image
Enzyme 1 GeneCard ID DHODH Link Image
Enzyme 1 GenAtlas ID DHODH Link Image
Enzyme 1 HGNC ID HGNC:2867 Link Image
Enzyme 1 Chromosome Location 16
Enzyme 1 Locus 16q22
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. Minet M, Dufour ME, Lacroute F: Cloning and sequencing of a human cDNA coding for dihydroorotate dehydrogenase by complementation of the corresponding yeast mutant. Gene. 1992 Nov 16;121(2):393-6. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 6334
Enzyme 2 Name Uridine 5'-monophosphate synthase
Enzyme 2 Synonyms
  1. UMP synthase[Includes: Orotate phosphoribosyltransferase
  2. OPRTase
  3. Orotidine 5'- phosphate decarboxylase
  4. OMPdecase]
Enzyme 2 Gene Name UMPS
Enzyme 2 Protein Sequence >Uridine 5'-monophosphate synthase 
MAVARAALGPLVTGLYDVQAFKFGDFVLKSGLSSPIYIDLRGIVSRPRLLSQVADILFQT
AQNAGISFDTVCGVPYTALPLATVICSTNQIPMLIRRKETKDYGTKRLVEGTINPGETCL
IIEDVVTSGSSVLETVEVLQKEGLKVTDAIVLLDREQGGKDKLQAHGIRLHSVCTLSKML
EILEQQKKVDAETVGRVKRFIQENVFVAANHNGSPLSIKEAPKELSFGARAELPRIHPVA
SKLLRLMQKKETNLCLSADVSLARELLQLADALGPSICMLKTHVDILNDFTLDVMKELIT
LAKCHEFLIFEDRKFADIGNTVKKQYEGGIFKIASWADLVNAHVVPGSGVVKGLQEVGLP
LHRGCLLIAEMSSTGSLATGDYTRAAVRMAEEHSEFVVGFISGSRVSMKPEFLHLTPGVQ
LEAGGDNLGQQYNSPQEVIGKRGSDIIIVGRGIISAADRLEAAEMYRKAAWEAYLSRLGV
Enzyme 2 Number of Residues 480
Enzyme 2 Molecular Weight 52222
Enzyme 2 Theoretical pI 7.26
Enzyme 2 GO Classification
Function
  • carbon-carbon lyase activity
  • carboxy-lyase activity
  • catalytic activity
  • lyase activity
  • orotate phosphoribosyltransferase activity
  • orotidine-5'-phosphate decarboxylase activity
  • transferase activity
  • transferase activity, transferring glycosyl groups
  • transferase activity, transferring pentosyl groups
Process
  • 'de novo' pyrimidine base biosynthesis
  • cellular metabolism
  • metabolism
  • nucleobase metabolism
  • nucleobase, nucleoside, nucleotide and nucleic acid metabolism
  • nucleoside metabolism
  • nucleotide metabolism
  • physiological process
  • pyrimidine base biosynthesis
  • pyrimidine base metabolism
  • pyrimidine nucleotide biosynthesis
  • pyrimidine nucleotide metabolism
Component
—
Enzyme 2 General Function Nucleotide transport and metabolism
Enzyme 2 Specific Function Orotidine 5'-phosphate + diphosphate = orotate + 5-phospho-alpha-D-ribose 1-diphosphate
Enzyme 2 Pathways
Enzyme 2 Reactions
  • orotidine 5'-phosphate = UMP + CO2
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • 1-20
Enzyme 2 Transmembrane Regions Not Available
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 340168 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID P11172 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name PYR5_HUMAN Link Image
Enzyme 2 PDB ID Not Available
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence >1443 bp 
ATGGCGGTCGCTCGTGCAGCTTTGGGGCCATTGGTGACGGGTCTGTACGACGTGCAGGCT
TTCAAGTTTGGGGACTTCGTGCTGAAGAGCGGGCTTTCCTCCCCCATCTACATCGATCTG
CGGGGCATCGTGTCTCGACCGCGTCTTCTGAGTCAGGTTGCAGATATTTTATTCCAAACT
GCCCAAAATGCAGGCATCAGTTTTGACACCGTGTGTGGAGTGCCTTATACAGCTTTGCCA
TTGGCTACAGTTATCTGTTCAACCAATCAAATTCCAATGCTTATTAGAAGGAAAGAAACA
AAGGATTATGGAACTAAGCGTCTTGTAGAAGGAACTATTAATCCAGGAGAAACCTGTTTA
ATCATTGAAGATGTTGTCACCAGTGGATCTAGTGTTTTGGAAACTGTTGAGGTTCTTCAG
AAGGAGGGCTTGAAGGTCACTGATGCCATAGTGCTGTTGGACAGAGAGCAGGGAGGCAAG
GACAAGTTGCAGGCGCACGGGATCCGCCTCCACTCAGTGTGTACATTGTCCAAAATGCTG
GAGATTCTCGAGCAGCAGAAAAAAGTTGATGCTGAGACAGTTGGGAGAGTGAAGAGGTTT
ATTCAGGAGAATGTCTTTGTGGCAGCGAATCATAATGGTTCTCCCCTTTCTATAAAGGAA
GCACCCAAAGAACTCAGCTTCGGTGCACGTGCAGAGCTGCCCAGGATCCACCCAGTTGCA
TCGAAGCTTCTCAGGCTTATGCAAAAGAAGGAGACCAATCTGTGTCTATCTGCTGATGTT
TCACTGGCCAGAGAGCTGTTGCAGCTAGCAGATGCTTTAGGACCTAGTATCTGCATGCTG
AAGACTCATGTAGATATTTTGAATGATTTTACTCTGGATGTGATGAAGGAGTTGATAACT
CTGGCAAAATGCCATGAGTTCTTGATATTTGAAGACCGGAAGTTTGCAGATATAGGAAAC
ACAGTGAAAAAGCAGTATGAAGGAGGTATCTTTAAAATAGCTTCCTGGGCAGATCTAGTA
AATGCTCACGTGGTGCCAGGCTCAGGAGTTGTGAAAGGCCTGCAAGAAGTGGGCCTGCCT
TTGCATCGGGGGTGCCTCCTTATTGCGGAAATGAGCTCCACCGGCTCCCTGGCCACTGGG
GACTACACTAGAGCAGCGGTTAGAATGGCTGAGGAGCACTCTGAATTTGTTGTTGGTTTT
ATTTCTGGCTCCCGAGTAAGCATGAAACCAGAATTTCTTCACTTGACTCCAGGAGTTCAG
TTGGAAGCAGGAGGAGATAATCTTGGCCAACAGTACAATAGCCCACAAGAAGTTATTGGC
AAACGAGGTTCCGATATCATCATTGTAGGTCGTGGCATAATCTCAGCAGCTGATCGTCTG
GAAGCAGCAGAGATGTACAGAAAAGCTGCTTGGGAAGCGTATTTGAGTAGACTTGGTGTT
TGA
Enzyme 2 GenBank Gene ID J03626 Link Image
Enzyme 2 GeneCard ID UMPS Link Image
Enzyme 2 GenAtlas ID UMPS Link Image
Enzyme 2 HGNC ID HGNC:12563 Link Image
Enzyme 2 Chromosome Location 3
Enzyme 2 Locus 3q13
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 General References
  1. Suttle DP, Bugg BY, Winkler JK, Kanalas JJ: Molecular cloning and nucleotide sequence for the complete coding region of human UMP synthase. Proc Natl Acad Sci U S A. 1988 Mar;85(6):1754-8. [PubMed Link Image]
  2. Suchi M, Harada N, Tsuboi T, Asai K, Okajima K, Wada Y, Takagi Y: Molecular cloning of human UMP synthase. Adv Exp Med Biol. 1989;253A:511-8. [PubMed Link Image]
  3. Suchi M, Mizuno H, Kawai Y, Tsuboi T, Sumi S, Okajima K, Hodgson ME, Ogawa H, Wada Y: Molecular cloning of the human UMP synthase gene and characterization of point mutations in two hereditary orotic aciduria families. Am J Hum Genet. 1997 Mar;60(3):525-39. [PubMed Link Image]
Enzyme 2 Metabolite References Not Available
Enzyme 3 [top]
Enzyme 3 ID 13096
Enzyme 3 Name cDNA FLJ75687, highly similar to Homo sapiens uridine monophosphate synthetase
Enzyme 3 Synonyms
  1. orotate phosphoribosyl transferase and orotidine-5'- decarboxylase
  2. UMPS, mRNA
  3. Uridine monophosphate synthetase
  4. Orotate phosphoribosyl transferase and orotidine-5'-decarboxylase, isoform CRA_b
Enzyme 3 Gene Name UMPS
Enzyme 3 Protein Sequence >cDNA FLJ75687, highly similar to Homo sapiens uridine monophosphate synthetase 
MAVARAALGPLVTGLYDVQAFKFGDFVLKSGLSSPIYIDLRGIVSRPRLLSQVADILFQT
AQNAGISFDTVCGVPYTALPLATVICSTNQIPMLIRRKETKDYGTKRLVEGTINPGETCL
IIEDVVTSGSSVLETVEVLQKEGLKVTDAIVLLDREQGGKDKLQAHGIRLHSVCTLSKML
EILEQQKKVDAETVGRVKRFIQENVFVAANHNGSPLSIKEAPKELSFGARAELPRIHPVA
SKLLRLMQKKETNLCLSADVSLARELLQLADALGPSICMLKTHVDILNDFTLDVMKELIT
LAKCHEFLIFEDRKFADIGNTVKKQYEGGIFKIASWADLVNAHVVPGSGVVKGLQEVGLP
LHRGCLLIAEMSSTGSLATGDYTRAAVRMAEEHSEFVVGFISGSRVSMKPEFLHLTPGVQ
LEAGGDNLGQQYNSPQEVIGKRGSDIIIVGRGIISAADRLEAAEMYRKAAWEAYLSRLGV
Enzyme 3 Number of Residues 480
Enzyme 3 Molecular Weight 52222
Enzyme 3 Theoretical pI 7.26
Enzyme 3 GO Classification Not Available
Enzyme 3 General Function Nucleotide transport and metabolism
Enzyme 3 Specific Function Not Available
Enzyme 3 Pathways Not Available
Enzyme 3 Reactions Not Available
Enzyme 3 Pfam Domain Function Not Available
Enzyme 3 Signals
  • None
Enzyme 3 Transmembrane Regions
  • None
Enzyme 3 Essentiality Not Available
Enzyme 3 GenBank ID Protein 158256048 Link Image
Enzyme 3 UniProtKB/Swiss-Prot ID A8K5J1 Link Image
Enzyme 3 UniProtKB/Swiss-Prot Entry Name A8K5J1_HUMAN Link Image
Enzyme 3 PDB ID Not Available
Enzyme 3 Cellular Location Not Available
Enzyme 3 Gene Sequence Not Available
Enzyme 3 GenBank Gene ID AK291306 Link Image
Enzyme 3 GeneCard ID A8K5J1 Link Image
Enzyme 3 GenAtlas ID Not Available
Enzyme 3 HGNC ID Not Available
Enzyme 3 Chromosome Location Not Available
Enzyme 3 Locus Not Available
Enzyme 3 SNPs SNPJam Report Link Image
Enzyme 3 General References Not Available
Enzyme 3 Metabolite References Not Available