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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-02-27 20:29:27 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
Data?1582752108
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
N-Carbamoyl-beta-alanineKegg
3-[(Aminocarbonyl)amino]propanoateGenerator
b-UreidopropionateGenerator
b-Ureidopropionic acidGenerator
beta-UreidopropionateGenerator
Β-ureidopropionateGenerator
Β-ureidopropionic acidGenerator
N-(AMINOCARBONYL)-b-alanineGenerator
N-(AMINOCARBONYL)-β-alanineGenerator
UreidopropanoateGenerator
N-Carbamoyl-b-alanineGenerator
N-Carbamoyl-β-alanineGenerator
UreidopropionateGenerator
3-(carbamoylamino)PropanoateHMDB
3-(carbamoylamino)Propanoic acidHMDB
3-ureido-PropionateHMDB
Carbamoyl-b-ala-OHHMDB
Carbamoyl-beta-ala-OHHMDB
N-(Aminocarbonyl)-'b-alanineHMDB
N-Carbamyl-b-alanineHMDB
N-Carbamyl-beta-alanineHMDB
Ion(-1) OF N-carbamoyl-beta-alanineMeSH, HMDB
Carbamoyl-beta-alanineHMDB
Carbamoyl-β-alanineHMDB
Carbamyl beta-alanineHMDB
Carbamyl β-alanineHMDB
beta-Ureidopropanoic acidHMDB
β-Ureidopropanoic acidHMDB
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
Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

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 Area92.42 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity28.82 m³·mol⁻¹ChemAxon
Polarizability12.07 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-002f-3910000000-bbd51b3bb30ff8d230d3Spectrum
GC-MSGC-MS Spectrum - GC-MS (1 TMS)splash10-00n0-5900000000-00dc280a63401c96ec2aSpectrum
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-0udi-2930000000-fcdce5cf4a20023c403aSpectrum
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-0hr0-2900000000-8a898fc59206b3f30a13Spectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-002f-3910000000-bbd51b3bb30ff8d230d3Spectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00n0-5900000000-00dc280a63401c96ec2aSpectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0udi-2930000000-fcdce5cf4a20023c403aSpectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0hr0-2900000000-8a898fc59206b3f30a13Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0udj-1910000000-83ab1eb4328f8caae993Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0v01-0900000000-aeacd84d8e0e91418232Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-006x-9000000000-f7cdf5fc49adf0b5e726Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00dr-9300000000-7b12845cd5bc4d02874cSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-00r6-9500000000-7ea8c95e3dc6296a07e9Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00di-9100000000-7b61b087fbf6c821bdd0Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0596-9200000000-79dfb7a1a6838745e178Spectrum
LC-MS/MSLC-MS/MS Spectrum - , positivesplash10-014l-8900000000-9bfe2297b9071d10d7e5Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-01b9-7900000000-684d9877d084bfc7c955Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00dl-9100000000-0b6f9ea311703e9ecf13Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-00b9-9000000000-1cb23a8265b882313309Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-001c-9300000000-2cec50bd5b226a288c82Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-000f-9100000000-63ecd2f9ef02c677503dSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0006-9000000000-396bc860a5f8f06ff7c5Spectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Biological Properties
Cellular Locations
  • Cytoplasm
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Locations
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
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
    FecesDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Normal
    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 Quantified<3.2 umol/mmol creatinineInfant (0-1 year old)Both
    Normal
    details
    UrineDetected and Quantified0.88 umol/mmol creatinineAdult (>18 years old)BothNormal details
    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 Quantified Adult (>18 years old)Both
    Colorectal cancer
    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 ]
    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 IDFDB030466
    KNApSAcK IDNot Available
    Chemspider ID109
    KEGG Compound IDC02642
    BioCyc ID3-UREIDO-PROPIONATE
    BiGG IDNot Available
    Wikipedia Link3-Ureidopropionic_acid
    METLIN IDNot Available
    PubChem Compound111
    PDB IDNot Available
    ChEBI ID18261
    Food Biomarker OntologyNot Available
    VMH 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)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 ]
    7. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

    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