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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:14:28 UTC
HMDB IDHMDB0000026
Secondary Accession Numbers
  • HMDB00026
  • HMDB0062812
  • HMDB62812
Metabolite Identification
Common NameUreidopropionic acid
DescriptionUreidopropionic acid, also known as 3-ureidopropanoate or N-carbamoyl-beta-alanine, belongs to the class of organic compounds known as ureas. Ureas are compounds containing two amine groups joined by a carbonyl (C=O) functional group. 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 into 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. Ureidopropionic acid has been detected, but not quantified in, several different foods, such as gram beans, broccoli, climbing beans, oriental wheat, and mandarin orange (clementine, tangerine). This could make ureidopropionic acid a potential biomarker for the consumption of these foods.
Structure
Thumb
Synonyms
Chemical FormulaC4H8N2O3
Average Molecular Weight132.1179
Monoisotopic Molecular Weight132.053492132
IUPAC Name3-(carbamoylamino)propanoic acid
Traditional Nameureidopropionic 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
Description Belongs to the class of organic compounds known as ureas. Ureas are compounds containing two amine groups joined by a carbonyl (C=O) functional group.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassOrganic carbonic acids and derivatives
Sub ClassUreas
Direct ParentUreas
Alternative Parents
Substituents
  • Urea
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effectNot Available
Disposition
Biological locationSource
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point170 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility20.9 mg/mLNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Locations
  • Placenta
Pathways
Normal Concentrations
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified0.5 (0.0-1.0) uMChildren (1-13 years old)BothDihydropyrimidinase deficiency details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer 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 ]
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  2. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB021879
KNApSAcK IDNot Available
Chemspider ID109
KEGG Compound IDC02642
BioCyc ID3-UREIDO-PROPIONATE
BiGG IDNot Available
Wikipedia Link3-Ureidopropionic_acid
METLIN IDNot Available
PubChem CompoundNot Available
PDB IDNot Available
ChEBI ID18261
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis Referencew-Ureido carboxylic acids. (1962), 3 pp. GB 913713 19621228 CAN 58:72975 AN 1963:72975
Material Safety Data Sheet (MSDS)Not Available
General References

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