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Human Metabolome Database Version 3.5

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Showing metabocard for Ureidopropionic acid (HMDB00026)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-02-08 17:07:46 -0700
HMDB ID HMDB00026
Secondary Accession Numbers None
Metabolite Identification
Common Name Ureidopropionic acid
Description Ureidopropionic acid is an intermediate in the metabolism of uracil. More specifically it is a breakdown product of dihydrouracil and is produced by the enzyme dihydropyrimidase. It is further decomposed to beta-alanine via the enzyme beta-ureidopropionase. Ureidopropionic acid is essentially a urea derivative of beta-alanine. High levels of Ureidopropionic acid are found in individuals with beta-ureidopropionase (UP) deficiency [PMID: 11675655 Link_out]. Enzyme deficiencies in pyrimidine metabolism are associated with a risk for severe toxicity against the antineoplastic agent 5-fluorouracil.
Structure Thumb
Download: MOL | SDF | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. 3-(Carbamoylamino)propanoate
  2. 3-(Carbamoylamino)propanoic acid
  3. 3-Ureido-propionate
  4. 3-Ureidopropanoate
  5. 3-Ureidopropanoic acid
  6. 3-Ureidopropionate
  7. 3-Ureidopropionic acid
  8. beta-Ureidopropionate
  9. beta-Ureidopropionic acid
  10. Carbamoyl-b-Ala-OH
  11. Carbamoyl-beta-Ala-OH
  12. N-(Aminocarbonyl)-'b-Alanine
  13. N-(Aminocarbonyl)-b-alanine
  14. N-(Aminocarbonyl)-beta-Alanine
  15. N-Carbamoyl-b-alanine
  16. N-Carbamoyl-beta-alanine
  17. N-Carbamyl-b-alanine
  18. N-Carbamyl-beta-alanine
  19. Ureidopropionate
  20. Ureidopropionic acid
Chemical Formula C4H8N2O3
Average Molecular Weight 132.1179
Monoisotopic Molecular Weight 132.053492132
IUPAC Name 3-(carbamoylamino)propanoic acid
Traditional IUPAC Name 3-ureidopropionic acid
CAS Registry Number 462-88-4
SMILES NC(=O)NCCC(O)=O
InChI Identifier InChI=1S/C4H8N2O3/c5-4(9)6-2-1-3(7)8/h1-2H2,(H,7,8)(H3,5,6,9)
InChI Key JSJWCHRYRHKBBW-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Aliphatic Acyclic Compounds
Class Alkylamines
Sub Class 1,3-Aminoalcohols
Other Descriptors
  • beta-alanine derivative(ChEBI)
Substituents
  • Carboxylic Acid
  • Urea
Direct Parent 1,3-Aminoalcohols
Ontology
Status Detected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Pantothenate and CoA biosynthesis
  • Component of Pyrimidine metabolism
  • Component of beta-Alanine metabolism
Application Not Available
Cellular locations
  • Cytoplasm
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 170 °C Not Available
Boiling Point Not Available Not Available
Water Solubility 20.9 mg/mL Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 52.7 g/L ALOGPS
LogP -0.98 ALOGPS
LogP -1.4 ChemAxon
LogS -0.40 ALOGPS
pKa (strongest acidic) 4.23 ChemAxon
pKa (strongest basic) -2 ChemAxon
Hydrogen Acceptor Count 3 ChemAxon
Hydrogen Donor Count 3 ChemAxon
Polar Surface Area 92.42 A2 ChemAxon
Rotatable Bond Count 3 ChemAxon
Refractivity 28.82 ChemAxon
Polarizability 12.07 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -1 ChemAxon
Spectra
1H NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
MS/MS Spectrum GC-MS
MS/MS Spectrum GC-MS
MS/MS Spectrum GC-MS
MS/MS Spectrum GC-MS
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location Not Available
Pathways
Name SMPDB Link KEGG Link
Beta-Alanine Metabolism SMP00007 map00410 Link_out
Pyrimidine Metabolism SMP00046 map00240 Link_out
Normal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Expected and not Quantified
Not Applicable Not Available Not Available Normal Inferred from detection in urine
Cerebrospinal Fluid (CSF) Detected and Quantified
Article_icon
0.0 - 0.5 uM Children (1-13 year old) Not Specified Normal Not Available
Urine Detected and Quantified
Article_icon
2.23 (1.48 - 2.81) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
Article_icon
2.09 (1.23–4.80) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
Article_icon
0.88 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Comments
Cerebrospinal Fluid (CSF) Detected and Quantified
Article_icon
0.5 (0.0-1.0) uM Children (1-13 year old) Both Dihydropyrimidinase deficiency Not Available
Associated Disorders and Diseases
Disease References
Dihydropyrimidinase deficiency
  • van Kuilenburg AB, Meijer J, Dobritzsch D, Meinsma R, Duran M, Lohkamp B, Zoetekouw L, Abeling NG, van Tinteren HL, Bosch AM: Clinical, biochemical and genetic findings in two siblings with a dihydropyrimidinase deficiency. Mol Genet Metab. 2007 Jun;91(2):157-64. Epub 2007 Mar 26. Pubmed: 17383919 Link_out
    Associated OMIM IDs
    • 222748 Link_out (Dihydropyrimidinase deficiency)
    DrugBank ID Not Available
    Phenol Explorer Compound ID Not Available
    Phenol Explorer Metabolite ID Not Available
    FoodDB ID FDB021879
    KNApSAcK ID Not Available
    Chemspider ID 109 Link_out
    KEGG Compound ID C02642 Link_out
    BioCyc ID 3-UREIDO-PROPIONATE Link_out
    BiGG ID 40270 Link_out
    Wikipedia Link Not Available
    NuGOwiki Link HMDB00026 Link_out
    Metagene Link HMDB00026 Link_out
    METLIN ID 5097 Link_out
    PubChem Compound 111 Link_out
    PDB ID URP Link_out
    ChEBI ID 18261 Link_out
    References
    Synthesis Reference w-Ureido carboxylic acids. (1962), 3 pp. GB 913713 19621228 CAN 58:72975 AN 1963:72975
    Material Safety Data Sheet (MSDS) Download (PDF)
    General References
    1. Moolenaar SH, Gohlich-Ratmann G, Engelke UF, Spraul M, Humpfer E, Dvortsak P, Voit T, Hoffmann GF, Brautigam C, van Kuilenburg AB, van Gennip A, Vreken P, Wevers RA: beta-Ureidopropionase deficiency: a novel inborn error of metabolism discovered using NMR spectroscopy on urine. Magn Reson Med. 2001 Nov;46(5):1014-7. Pubmed: 11675655 Link_out
    2. Sparidans RW, Bosch TM, Jorger M, Schellens JH, Beijnen JH: Liquid chromatography-tandem mass spectrometric assay for the analysis of uracil, 5,6-dihydrouracil and beta-ureidopropionic acid in urine for the measurement of the activities of the pyrimidine catabolic enzymes. J Chromatogr B Analyt Technol Biomed Life Sci. 2006 Jul 24;839(1-2):45-53. Epub 2006 Feb 28. Pubmed: 16513432 Link_out
    3. Ito S, Kawamura T, Inada M, Inoue Y, Hirao Y, Koga T, Kunizaki J, Shimizu T, Sato H: Physiologically based pharmacokinetic modelling of the three-step metabolism of pyrimidine using C-uracil as an in vivo probe. Br J Clin Pharmacol. 2005 Dec;60(6):584-93. Pubmed: 16305582 Link_out
    4. Hofmann U, Schwab M, Seefried S, Marx C, Zanger UM, Eichelbaum M, Murdter TE: Sensitive method for the quantification of urinary pyrimidine metabolites in healthy adults by gas chromatography-tandem mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 5;791(1-2):371-80. Pubmed: 12798197 Link_out
    5. Malet-Martino MC, Armand JP, Lopez A, Bernadou J, Beteille JP, Bon M, Martino R: Evidence for the importance of 5'-deoxy-5-fluorouridine catabolism in humans from 19F nuclear magnetic resonance spectrometry. Cancer Res. 1986 Apr;46(4 Pt 2):2105-12. Pubmed: 2936452 Link_out
    6. Desmoulin F, Gilard V, Malet-Martino M, Martino R: Metabolism of capecitabine, an oral fluorouracil prodrug: (19)F NMR studies in animal models and human urine. Drug Metab Dispos. 2002 Nov;30(11):1221-9. Pubmed: 12386128 Link_out
    Enzymes
    Name: Dihydropyrimidinase
    Reactions:
    • 5,6-dihydrouracil + H2O = 3-ureidopropanoate [RN:R02269]
    Gene Name: DPYS
    Uniprot ID: Q14117 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Beta-ureidopropionase
    Reactions:
    • N-carbamoyl-beta-alanine + H2O = beta-alanine + CO2 + NH3 [RN:R00905]
    Gene Name: UPB1
    Uniprot ID: Q9UBR1 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Dihydropyrimidinase-related protein 3
    Reactions:
      Gene Name: DPYSL3
      Uniprot ID: Q14195 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: Dihydropyrimidinase-related protein 2
      Reactions:
        Gene Name: DPYSL2
        Uniprot ID: Q16555 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA