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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2006-05-22 14:17:33 UTC
Update Date2021-09-14 15:20:03 UTC
HMDB IDHMDB0002031
Secondary Accession Numbers
  • HMDB02031
Metabolite Identification
Common NameUreidoisobutyric acid
DescriptionUreidoisobutyric acid, also known as 3-ureidoisobutyrate or beta-UBA, 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. Ureidoisobutyric acid is an extremely weak basic (essentially neutral) compound (based on its pKa). Ureidoisobutyric acid exists in all living organisms, ranging from bacteria to humans. Within humans, ureidoisobutyric acid participates in a number of enzymatic reactions. In particular, ureidoisobutyric acid can be biosynthesized from dihydrothymine through its interaction with the enzyme dihydropyrimidinase. Outside of the human body, ureidoisobutyric acid has been detected, but not quantified in, several different foods, such as bread, squashberries, black elderberries, black crowberries, and climbing beans. This could make ureidoisobutyric acid a potential biomarker for the consumption of these foods. Ureidoisobutyric acid is increased in the urine of patients with beta-ureidopropionase (EC 3.5.1.6) deficiency (PMID: 12271438 ), a genetic disorder. Ureidoisobutyric acid can be used to predict a patient's individual phenotypes of enzyme deficiencies in pyrimidine metabolism when associated with a risk for severe toxicity against the antineoplastic agent 5-fluorouracil (PMID: 12798197 ).
Structure
Data?1583853784
Synonyms
ValueSource
UreidoisobutyrateGenerator
(2S)-3-[(C-Hydroxycarbonimidoyl)amino]-2-methylpropanoateHMDB
(2S)-3-(Carbamoylamino)-2-methylpropanoic acidHMDB
(2S)-3-(Carbamoylamino)-2-methylpropionic acidHMDB
3-((Aminocarbonyl)amino)-2-methylpropanoic acidHMDB
3-((Aminocarbonyl)amino)-2-methylpropionic acidHMDB
3-UreidoisobutyrateHMDB
3-[(Aminocarbonyl)amino]-2-methylpropanoic acidHMDB
3-[(Aminocarbonyl)amino]-2-methylpropionic acidHMDB
beta-UBAHMDB
beta-Ureidoisobutyric acidHMDB
Β-ubaHMDB
Β-ureidoisobutyric acidHMDB
Chemical FormulaC5H10N2O3
Average Molecular Weight146.1445
Monoisotopic Molecular Weight146.069142196
IUPAC Name(2S)-3-(carbamoylamino)-2-methylpropanoic acid
Traditional Name(2S)-3-(carbamoylamino)-2-methylpropanoic acid
CAS Registry Number19140-82-0
SMILES
C[C@@H](CNC(N)=O)C(O)=O
InChI Identifier
InChI=1S/C5H10N2O3/c1-3(4(8)9)2-7-5(6)10/h3H,2H2,1H3,(H,8,9)(H3,6,7,10)/t3-/m0/s1
InChI KeyPHENTZNALBMCQD-VKHMYHEASA-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
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
Physiological effectNot Available
Disposition
Biological locationSource
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility46.1 g/LALOGPS
logP-0.86ALOGPS
logP-0.88ChemAxon
logS-0.5ALOGPS
pKa (Strongest Acidic)4.32ChemAxon
pKa (Strongest Basic)-1.8ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area92.42 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity33.4 m³·mol⁻¹ChemAxon
Polarizability13.82 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DeepCCS[M+H]+125.75530932474
DeepCCS[M-H]-121.8930932474
DeepCCS[M-2H]-159.25230932474
DeepCCS[M+Na]+134.6930932474

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
Ureidoisobutyric acidC[C@@H](CNC(N)=O)C(O)=O2299.5Standard polar33892256
Ureidoisobutyric acidC[C@@H](CNC(N)=O)C(O)=O1431.4Standard non polar33892256
Ureidoisobutyric acidC[C@@H](CNC(N)=O)C(O)=O1733.4Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Ureidoisobutyric acid,1TMS,isomer #1C[C@@H](CNC(N)=O)C(=O)O[Si](C)(C)C1584.3Semi standard non polar33892256
Ureidoisobutyric acid,1TMS,isomer #2C[C@@H](CNC(=O)N[Si](C)(C)C)C(=O)O1622.7Semi standard non polar33892256
Ureidoisobutyric acid,1TMS,isomer #3C[C@@H](CN(C(N)=O)[Si](C)(C)C)C(=O)O1596.3Semi standard non polar33892256
Ureidoisobutyric acid,2TMS,isomer #1C[C@@H](CNC(=O)N[Si](C)(C)C)C(=O)O[Si](C)(C)C1673.6Semi standard non polar33892256
Ureidoisobutyric acid,2TMS,isomer #1C[C@@H](CNC(=O)N[Si](C)(C)C)C(=O)O[Si](C)(C)C1477.9Standard non polar33892256
Ureidoisobutyric acid,2TMS,isomer #1C[C@@H](CNC(=O)N[Si](C)(C)C)C(=O)O[Si](C)(C)C2225.5Standard polar33892256
Ureidoisobutyric acid,2TMS,isomer #2C[C@@H](CN(C(N)=O)[Si](C)(C)C)C(=O)O[Si](C)(C)C1570.2Semi standard non polar33892256
Ureidoisobutyric acid,2TMS,isomer #2C[C@@H](CN(C(N)=O)[Si](C)(C)C)C(=O)O[Si](C)(C)C1550.0Standard non polar33892256
Ureidoisobutyric acid,2TMS,isomer #2C[C@@H](CN(C(N)=O)[Si](C)(C)C)C(=O)O[Si](C)(C)C2370.4Standard polar33892256
Ureidoisobutyric acid,2TMS,isomer #3C[C@@H](CN(C(=O)N[Si](C)(C)C)[Si](C)(C)C)C(=O)O1660.4Semi standard non polar33892256
Ureidoisobutyric acid,2TMS,isomer #3C[C@@H](CN(C(=O)N[Si](C)(C)C)[Si](C)(C)C)C(=O)O1612.0Standard non polar33892256
Ureidoisobutyric acid,2TMS,isomer #3C[C@@H](CN(C(=O)N[Si](C)(C)C)[Si](C)(C)C)C(=O)O2257.0Standard polar33892256
Ureidoisobutyric acid,2TMS,isomer #4C[C@@H](CNC(=O)N([Si](C)(C)C)[Si](C)(C)C)C(=O)O1738.2Semi standard non polar33892256
Ureidoisobutyric acid,2TMS,isomer #4C[C@@H](CNC(=O)N([Si](C)(C)C)[Si](C)(C)C)C(=O)O1558.2Standard non polar33892256
Ureidoisobutyric acid,2TMS,isomer #4C[C@@H](CNC(=O)N([Si](C)(C)C)[Si](C)(C)C)C(=O)O2347.8Standard polar33892256
Ureidoisobutyric acid,3TMS,isomer #1C[C@@H](CN(C(=O)N[Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C1664.8Semi standard non polar33892256
Ureidoisobutyric acid,3TMS,isomer #1C[C@@H](CN(C(=O)N[Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C1588.8Standard non polar33892256
Ureidoisobutyric acid,3TMS,isomer #1C[C@@H](CN(C(=O)N[Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C1906.1Standard polar33892256
Ureidoisobutyric acid,3TMS,isomer #2C[C@@H](CNC(=O)N([Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C1716.2Semi standard non polar33892256
Ureidoisobutyric acid,3TMS,isomer #2C[C@@H](CNC(=O)N([Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C1597.4Standard non polar33892256
Ureidoisobutyric acid,3TMS,isomer #2C[C@@H](CNC(=O)N([Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C1974.5Standard polar33892256
Ureidoisobutyric acid,3TMS,isomer #3C[C@@H](CN(C(=O)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C)C(=O)O1730.8Semi standard non polar33892256
Ureidoisobutyric acid,3TMS,isomer #3C[C@@H](CN(C(=O)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C)C(=O)O1741.1Standard non polar33892256
Ureidoisobutyric acid,3TMS,isomer #3C[C@@H](CN(C(=O)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C)C(=O)O1996.4Standard polar33892256
Ureidoisobutyric acid,4TMS,isomer #1C[C@@H](CN(C(=O)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C1765.1Semi standard non polar33892256
Ureidoisobutyric acid,4TMS,isomer #1C[C@@H](CN(C(=O)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C1757.2Standard non polar33892256
Ureidoisobutyric acid,4TMS,isomer #1C[C@@H](CN(C(=O)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C)C(=O)O[Si](C)(C)C1711.6Standard polar33892256
Ureidoisobutyric acid,1TBDMS,isomer #1C[C@@H](CNC(N)=O)C(=O)O[Si](C)(C)C(C)(C)C1836.8Semi standard non polar33892256
Ureidoisobutyric acid,1TBDMS,isomer #2C[C@@H](CNC(=O)N[Si](C)(C)C(C)(C)C)C(=O)O1894.6Semi standard non polar33892256
Ureidoisobutyric acid,1TBDMS,isomer #3C[C@@H](CN(C(N)=O)[Si](C)(C)C(C)(C)C)C(=O)O1833.6Semi standard non polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #1C[C@@H](CNC(=O)N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2120.7Semi standard non polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #1C[C@@H](CNC(=O)N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1890.5Standard non polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #1C[C@@H](CNC(=O)N[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2274.3Standard polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #2C[C@@H](CN(C(N)=O)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2030.3Semi standard non polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #2C[C@@H](CN(C(N)=O)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1976.5Standard non polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #2C[C@@H](CN(C(N)=O)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2477.5Standard polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #3C[C@@H](CN(C(=O)N[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O2123.1Semi standard non polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #3C[C@@H](CN(C(=O)N[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O1993.1Standard non polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #3C[C@@H](CN(C(=O)N[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O2327.8Standard polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #4C[C@@H](CNC(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O2164.2Semi standard non polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #4C[C@@H](CNC(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O1981.5Standard non polar33892256
Ureidoisobutyric acid,2TBDMS,isomer #4C[C@@H](CNC(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O2351.2Standard polar33892256
Ureidoisobutyric acid,3TBDMS,isomer #1C[C@@H](CN(C(=O)N[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2325.1Semi standard non polar33892256
Ureidoisobutyric acid,3TBDMS,isomer #1C[C@@H](CN(C(=O)N[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2208.7Standard non polar33892256
Ureidoisobutyric acid,3TBDMS,isomer #1C[C@@H](CN(C(=O)N[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2243.4Standard polar33892256
Ureidoisobutyric acid,3TBDMS,isomer #2C[C@@H](CNC(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2352.5Semi standard non polar33892256
Ureidoisobutyric acid,3TBDMS,isomer #2C[C@@H](CNC(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2233.1Standard non polar33892256
Ureidoisobutyric acid,3TBDMS,isomer #2C[C@@H](CNC(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2248.5Standard polar33892256
Ureidoisobutyric acid,3TBDMS,isomer #3C[C@@H](CN(C(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O2384.0Semi standard non polar33892256
Ureidoisobutyric acid,3TBDMS,isomer #3C[C@@H](CN(C(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O2313.1Standard non polar33892256
Ureidoisobutyric acid,3TBDMS,isomer #3C[C@@H](CN(C(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O2289.0Standard polar33892256
Ureidoisobutyric acid,4TBDMS,isomer #1C[C@@H](CN(C(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2599.9Semi standard non polar33892256
Ureidoisobutyric acid,4TBDMS,isomer #1C[C@@H](CN(C(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2520.8Standard non polar33892256
Ureidoisobutyric acid,4TBDMS,isomer #1C[C@@H](CN(C(=O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2215.7Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - Ureidoisobutyric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 10V, Positive-QTOFNot Available2020-06-30Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 20V, Positive-QTOFNot Available2020-06-30Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 40V, Positive-QTOFNot Available2020-06-30Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 10V, Negative-QTOFNot Available2020-06-30Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 20V, Negative-QTOFNot Available2020-06-30Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 40V, Negative-QTOFNot Available2020-06-30Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 10V, Positive-QTOFsplash10-0019-8900000000-51286b64a41bad89fd802021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 20V, Positive-QTOFsplash10-05gi-9100000000-835c9b02eecbd89baac82021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 40V, Positive-QTOFsplash10-0006-9000000000-40f2b4e3a071943603ee2021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 10V, Negative-QTOFsplash10-0udi-3900000000-9dfc177176195ee471922021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 20V, Negative-QTOFsplash10-052f-9000000000-0f56471f86b7f22306732021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ureidoisobutyric acid 40V, Negative-QTOFsplash10-0006-9000000000-60a7db0ec3c9213e93472021-09-24Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-25Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot QuantifiedNot AvailableNot Available
Normal
      Not Available
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.01 +/- 0.05 uMAdult (>18 years old)BothNormal details
UrineDetected and Quantified<0.9 umol/mmol creatinineInfant (0-1 year old)Both
Normal
details
UrineDetected and Quantified0.113 umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified1.4 - 5.9 uMAdult (>18 years old)BothBeta-ureidopropionase deficiency details
Associated Disorders and Diseases
Disease References
Beta-ureidopropionase deficiency
  1. van Kuilenburg AB, Meinsma R, Beke E, Assmann B, Ribes A, Lorente I, Busch R, Mayatepek E, Abeling NG, van Cruchten A, Stroomer AE, van Lenthe H, Zoetekouw L, Kulik W, Hoffmann GF, Voit T, Wevers RA, Rutsch F, van Gennip AH: beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities. Hum Mol Genet. 2004 Nov 15;13(22):2793-801. Epub 2004 Sep 22. [PubMed:15385443 ]
Associated OMIM IDs
  • 613161 (Beta-ureidopropionase deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022808
KNApSAcK IDNot Available
Chemspider IDNot Available
KEGG Compound IDC05100
BioCyc ID3-UREIDO-ISOBUTYRATE
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound88558720
PDB IDURQ
ChEBI IDNot Available
Food Biomarker OntologyNot Available
VMH ID3UIB
MarkerDB IDMDB00000369
Good Scents IDNot Available
References
Synthesis ReferenceEvans, W. R.; Tsai, Chi Shiun; Axelrod, Bernard. Origin of b-aminoisobutyric acid in iris. Nature (London, United Kingdom) (1961), 190 809.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. 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 ]
  2. Ohse M, Matsuo M, Ishida A, Kuhara T: Screening and diagnosis of beta-ureidopropionase deficiency by gas chromatographic/mass spectrometric analysis of urine. J Mass Spectrom. 2002 Sep;37(9):954-62. [PubMed:12271438 ]

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
Dihydrothymine + Water → Ureidoisobutyric 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
Ureidoisobutyric acid + Water → 3-Aminoisobutanoic acid + Carbon dioxide + Ammoniadetails