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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-08-16 03:09:37 UTC
HMDB IDHMDB0000026
Secondary Accession Numbers
  • HMDB00026
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
ValueSource
3-[(Aminocarbonyl)amino]propanoic acidChEBI
3-UreidopropanoateChEBI
3-Ureidopropanoic acidChEBI
3-UreidopropionateChEBI
3-Ureidopropionic acidChEBI
beta-Ureidopropionic acidChEBI
N-(AMINOCARBONYL)-BETA-alanineChEBI
Ureidopropanoic acidChEBI
3-[(Aminocarbonyl)amino]propanoateGenerator
UreidopropionateGenerator
b-UreidopropionateGenerator
b-Ureidopropionic acidGenerator
beta-UreidopropionateGenerator
β-ureidopropionateGenerator
β-ureidopropionic acidGenerator
N-(AMINOCARBONYL)-b-alanineGenerator
N-(AMINOCARBONYL)-β-alanineGenerator
UreidopropanoateGenerator
3-(carbamoylamino)PropanoateHMDB
3-(carbamoylamino)Propanoic acidHMDB
3-ureido-PropionateHMDB
Carbamoyl-b-ala-OHHMDB
Carbamoyl-beta-ala-OHHMDB
N-(Aminocarbonyl)-'b-alanineHMDB
N-Carbamoyl-b-alanineHMDB
N-Carbamoyl-beta-alanineHMDB
N-Carbamyl-b-alanineHMDB
N-Carbamyl-beta-alanineHMDB
Ion(-1) OF N-carbamoyl-beta-alanineMeSH
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
DescriptionThis compound belongs to the class of chemical entities known as ureas. These are compounds containing two amine groups joined by a carbonyl (C=O) functional group.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganic acids and derivatives
Sub ClassOrganic carbonic acids and derivatives
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
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 mg/mLALOGPS
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 Area92.42 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity28.82 m3·mol-1ChemAxon
Polarizability12.07 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MSsplash10-002f-3910000000-bbd51b3bb30ff8d230d3View in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 TMS)splash10-00n0-5900000000-00dc280a63401c96ec2aView in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-0udi-2930000000-fcdce5cf4a20023c403aView in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-0hr0-2900000000-8a898fc59206b3f30a13View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOFsplash10-0udj-1910000000-83ab1eb4328f8caae993View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOFsplash10-0udj-1910000000-83ab1eb4328f8caae993View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOFsplash10-0v01-0900000000-aeacd84d8e0e91418232View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-00r6-9500000000-7ea8c95e3dc6296a07e9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00di-9100000000-7b61b087fbf6c821bdd0View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0596-9200000000-79dfb7a1a6838745e178View in MoNA
LC-MS/MSLC-MS/MS Spectrum - , positivesplash10-014l-8900000000-9bfe2297b9071d10d7e5View in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Beta Ureidopropionase DeficiencySMP00172Not Available
Beta-Alanine MetabolismSMP00007map00410
Carnosinuria, carnosinemiaSMP00493Not Available
Dihydropyrimidinase DeficiencySMP00178Not Available
GABA-Transaminase DeficiencySMP00351Not Available
MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy)SMP00202Not Available
Pyrimidine MetabolismSMP00046map00240
UMP Synthase Deiciency (Orotic Aciduria)SMP00219Not Available
Ureidopropionase deficiencySMP00492Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not Quantified Not AvailableNot Available
Normal
    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
    FecesDetected but not Quantified Adult (>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 ]
    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 LinkHMDB0000026
    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