You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version3.6
Creation Date2006-05-22 14:17:33 UTC
Update Date2016-02-11 01:05:14 UTC
HMDB IDHMDB02031
Secondary Accession NumbersNone
Metabolite Identification
Common NameUreidoisobutyric acid
DescriptionBeta-ureidoisobutyrate (betaUIB) is increased in the urine of patients with beta-ureidopropionase deficiency [EC 3.5.1.6] (PMID: 12271438 ). Ureidoisobutyric acid can be used to predict 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
Thumb
Synonyms
ValueSource
3-((Aminocarbonyl)amino)-2-methylpropanoic acidChEBI
3-UreidoisobutyrateChEBI
beta-UBAChEBI
beta-Ureidoisobutyric acidChEBI
3-((Aminocarbonyl)amino)-2-methylpropanoateGenerator
b-UBAGenerator
β-ubaGenerator
b-UreidoisobutyrateGenerator
b-Ureidoisobutyric acidGenerator
beta-UreidoisobutyrateGenerator
β-ureidoisobutyrateGenerator
β-ureidoisobutyric acidGenerator
3-carbamoylamino-2-MethylpropanoateHMDB
3-carbamoylamino-2-Methylpropanoic acidHMDB
DL-beta-UreidoisobutyrateHMDB
DL-beta-Ureidoisobutyric acidHMDB
N-Carbamyl-amino isobutyrateHMDB
N-Carbamyl-b-aminoisobutyric acidHMDB
N-Carbamyl-beta-aminoisobutyric acidHMDB
Chemical FormulaC5H10N2O3
Average Molecular Weight146.1445
Monoisotopic Molecular Weight146.069142196
IUPAC Name3-(carbamoylamino)-2-methylpropanoic acid
Traditional Nameβ-ureidoisobutyric acid
CAS Registry Number2905-86-4
SMILES
CC(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)
InChI KeyInChIKey=PHENTZNALBMCQD-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as ureas. These 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
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Protein synthesis, amino acid biosynthesis
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility46.1 mg/mLALOGPS
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 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity33.4 m3·mol-1ChemAxon
Polarizability13.81 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0ug1-4900000000-57fe49b6570a3a56075eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000i-9400000000-d75bc53f6bd893972265View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9000000000-505e1e587ff8e3f47c4bView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0udj-6900000000-d0e112adeac57b5cc676View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0zfu-9600000000-38cc35413fc058ff2f4fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0006-9000000000-46efbc5db1bc9c62db04View in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Beta Ureidopropionase DeficiencySMP00172Not Available
Dihydropyrimidinase DeficiencySMP00178Not Available
MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy)SMP00202Not Available
Pyrimidine MetabolismSMP00046map00240
UMP Synthase Deiciency (Orotic Aciduria)SMP00219Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot ApplicableNot AvailableNot Available
Normal
  • Not Applicable
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.01 +/- 0.05 uMAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.113 umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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. [15385443 ]
Associated OMIM IDs
  • 613161 (Beta-ureidopropionase deficiency)
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022808
KNApSAcK IDNot Available
Chemspider ID141172
KEGG Compound IDC05100
BioCyc ID3-UREIDO-ISOBUTYRATE
BiGG ID45118
Wikipedia LinkNot Available
NuGOwiki LinkHMDB02031
Metagene LinkHMDB02031
METLIN IDNot Available
PubChem Compound160663
PDB IDNot Available
ChEBI ID1670
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. [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. [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