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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2013-05-29 19:24:29 UTC
HMDB IDHMDB00026
Secondary Accession NumbersNone
Metabolite Identification
Common NameUreidopropionic acid
DescriptionUreidopropionic 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 ]. Enzyme deficiencies in pyrimidine metabolism are associated with a risk for severe toxicity against the antineoplastic agent 5-fluorouracil.
Structure
Thumb
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 FormulaC4H8N2O3
Average Molecular Weight132.1179
Monoisotopic Molecular Weight132.053492132
IUPAC Name3-(carbamoylamino)propanoic acid
Traditional IUPAC Name3-ureidopropionic acid
CAS Registry Number462-88-4
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 KeyJSJWCHRYRHKBBW-UHFFFAOYSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassAliphatic Acyclic Compounds
ClassAlkylamines
Sub Class1,3-Aminoalcohols
Other Descriptors
  • beta-alanine derivative(ChEBI)
Substituents
  • Carboxylic Acid
  • Urea
Direct Parent1,3-Aminoalcohols
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Pantothenate and CoA biosynthesis
  • Component of Pyrimidine metabolism
  • Component of beta-Alanine metabolism
ApplicationNot Available
Cellular locations
  • Cytoplasm
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point170 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility20.9 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility52.7 g/LALOGPS
logP-0.98ALOGPS
logP-1.4ChemAxon
logS-0.4ALOGPS
pKa (Strongest Acidic)4.23ChemAxon
pKa (Strongest Basic)-2ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area92.42ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity28.82ChemAxon
Polarizability12.07ChemAxon
Spectra
SpectraGC-MSMS/MS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Cytoplasm
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Beta-Alanine MetabolismSMP00007map00410
Pyrimidine MetabolismSMP00046map00240
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot ApplicableNot AvailableNot Available
Normal
  • Not Applicable
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.0 - 0.5 uMChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified2.23 (1.48 - 2.81) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified2.09 (1.23–4.80) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.88 umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified0.5 (0.0-1.0) uMChildren (1-13 years old)BothDihydropyrimidinase deficiency details
Associated Disorders and Diseases
Disease References
Dihydropyrimidinase deficiency
  1. 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
Associated OMIM IDs
  • 222748 (Dihydropyrimidinase deficiency)
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB021879
KNApSAcK IDNot Available
Chemspider ID109
KEGG Compound IDC02642
BioCyc ID3-UREIDO-PROPIONATE
BiGG ID40270
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00026
Metagene LinkHMDB00026
METLIN ID5097
PubChem Compound111
PDB IDURP
ChEBI ID18261
References
Synthesis Referencew-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
  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
  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
  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
  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
  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

Enzymes

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.
Gene Name:
DPYS
Uniprot ID:
Q14117
Molecular weight:
56629.36
Reactions
Dihydrouracil + Water → Ureidopropionic aciddetails
General function:
Involved in hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds
Specific function:
Converts N-carbamyl-beta-aminoisobutyric acid and N-carbamyl-beta-alanine to, respectively, beta-aminoisobutyric acid and beta-alanine, ammonia and carbon dioxide.
Gene Name:
UPB1
Uniprot ID:
Q9UBR1
Molecular weight:
43165.705
Reactions
Ureidopropionic acid + Water → Beta-Alanine + CO(2) + Ammoniadetails
Ureidopropionic acid + Water → Beta-Alanine + Carbon dioxide + Ammoniadetails
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
General function:
Involved in hydrolase activity
Specific function:
Necessary for signaling by class 3 semaphorins and subsequent remodeling of the cytoskeleton. Plays a role in axon guidance, neuronal growth cone collapse and cell migration
Gene Name:
DPYSL3
Uniprot ID:
Q14195
Molecular weight:
61962.8
General function:
Nucleotide transport and metabolism
Specific function:
Necessary for signaling by class 3 semaphorins and subsequent remodeling of the cytoskeleton. Plays a role in axon guidance, neuronal growth cone collapse and cell migration
Gene Name:
DPYSL2
Uniprot ID:
Q16555
Molecular weight:
62293.1