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Record Information
Version3.6
Creation Date2006-05-22 14:17:39 UTC
Update Date2016-02-11 01:05:24 UTC
HMDB IDHMDB02166
Secondary Accession NumbersNone
Metabolite Identification
Common Name(S)-b-aminoisobutyric acid
DescriptionBeta-Aminoisobutyric acid is a non-protein amino acid originating from the catabolism of thymine and valine. The concentration of beta-Aminoisobutyric acid is normally low in urine as beta-Aminoisobutyric acid is further catabolized by b-aminoisobutyrate aminotransferases to methylmalonic acid semialdehyde and propionyl-CoA. beta-Aminoisobutyric acid occurs in two isomeric forms and both enantiomers of beta-Aminoisobutyric acid can be detected in human urine and plasma. In plasma, the S-enantiomer is the predominant type due to active renal reabsorption. In contrast, urine almost exclusively contains the R-enantiomer of beta-Aminoisobutyric acid, which is eliminated both by filtration and tubular secretion. Persistently increased levels of beta-Aminoisobutyric acid have been observed in individuals with a deficiency of R (-) -b-aminoisobutyrate-pyruvate aminotransferase. In addition, transient high levels of beta-Aminoisobutyric acid have been observed under a variety of pathological conditions such as lead poisoning, starvation, in total body irradiation and in a number of malignancies. The S-enantiomer of beta-Aminoisobutyric acid is predominantly derived from the catabolism of valine. It has been suggested that an altered homoeostasis of b-alanine underlies some of the clinical abnormalities encountered in patients with a dihydropyrimidine dehydrogenase (DPD) deficiency. DPD constitutes the first step of the pyrimidine degradation pathway, in which the pyrimidine bases uracil and thymine are catabolized to b-alanine and the R-enantiomer of beta-Aminoisobutyric acid respectively. In normal individuals with an intact pyrimidine degradation pathway, R-methylmalonic acid semialdehyde can be synthesized directly from the catabolism of thymine. Hence, there might be less cross-over between the valine and thymine pathway, allowing the conversion of S-methylmalonic acid semialdehyde into S-beta-Aminoisobutyric acid and the subsequent accumulation of S-beta-Aminoisobutyric acid in plasma. (PMID: 14705962 , 14292857 , 14453202 ).
Structure
Thumb
Synonyms
ValueSource
(S)-3-amino-2-Methylpropanoic acidChEBI
(S)-3-amino-Isobutanoic acidChEBI
(S)-3-amino-Isobutyric acidChEBI
(S)-beta-Aminoisobutyric acidChEBI
L-3-amino-Isobutanoic acidChEBI
L-3-amino-Isobutyric acidChEBI
(S)-3-amino-2-MethylpropanoateGenerator
(S)-3-AminoisobutyrateGenerator
(S)-3-amino-IsobutanoateGenerator
(S)-3-amino-IsobutyrateGenerator
(S)-b-AminoisobutyrateGenerator
(S)-b-Aminoisobutyric acidGenerator
(S)-beta-AminoisobutyrateGenerator
(S)-β-aminoisobutyrateGenerator
(S)-β-aminoisobutyric acidGenerator
L-3-amino-IsobutanoateGenerator
L-3-amino-IsobutyrateGenerator
(+)-a-Methyl-b-alanineHMDB
(+)-alpha-Methyl-beta-alanineHMDB
(+)-b-Aminoisobutyric acidHMDB
(+)-beta-Aminoisobutyric acidHMDB
(S)-3-amino-2-Methyl-propanoateHMDB
(S)-3-amino-2-Methyl-propanoic acidHMDB
L-2-Methyl-b-alanineHMDB
L-2-Methyl-beta-alanineHMDB
L-3-amino-2-MethylpropanoateHMDB
L-3-amino-2-Methylpropanoic acidHMDB
L-3-amino-2-Methylpropionic acidHMDB
L-b-AminoisobutyrateHMDB
L-b-Aminoisobutyric acidHMDB
L-beta-AminoisobutyrateHMDB
L-beta-Aminoisobutyric acidHMDB
S-b-AminoisobutyrateHMDB
S-beta-AminoisobutyrateHMDB
S-beta-Aminoisobutyric acidHMDB
Chemical FormulaC4H9NO2
Average Molecular Weight103.1198
Monoisotopic Molecular Weight103.063328537
IUPAC Name(2S)-3-amino-2-methylpropanoic acid
Traditional Name(+)-α-methyl-β-alanine
CAS Registry Number4249-19-8
SMILES
C[C@@H](CN)C(O)=O
InChI Identifier
InChI=1S/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)/t3-/m0/s1
InChI KeyInChIKey=QCHPKSFMDHPSNR-VKHMYHEASA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as beta amino acids and derivatives. These are amino acids having a (-NH2) group attached to the beta carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentBeta amino acids and derivatives
Alternative Parents
Substituents
  • Beta amino acid or derivatives
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Protein synthesis, amino acid biosynthesis
ApplicationNot Available
Cellular locationsNot Available
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point175 - 177 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility367.0 mg/mLALOGPS
logP-3ALOGPS
logP-2.6ChemAxon
logS0.55ALOGPS
pKa (Strongest Acidic)4.17ChemAxon
pKa (Strongest Basic)10.32ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity25.28 m3·mol-1ChemAxon
Polarizability10.42 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, Positivesplash10-0f79-9300000000-30669ed04aadf3d3f040View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000l-9000000000-ca65637476e64152c44eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9000000000-04d1648391d3f90db226View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0udi-3900000000-ec54fcce079024a62344View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0zfr-9500000000-0394f399cdf5df93c5dfView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4l-9000000000-d0c495c908ed905558a2View in MoNA
Biological Properties
Cellular LocationsNot Available
Biofluid Locations
  • Blood
  • Feces
  • Urine
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase DeficiencySMP00137Not Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencySMP00138Not Available
3-hydroxyisobutyric acid dehydrogenase deficiencySMP00521Not Available
3-hydroxyisobutyric aciduriaSMP00522Not Available
3-Methylcrotonyl Coa Carboxylase Deficiency Type ISMP00237Not Available
3-Methylglutaconic Aciduria Type ISMP00139Not Available
3-Methylglutaconic Aciduria Type IIISMP00140Not Available
3-Methylglutaconic Aciduria Type IVSMP00141Not Available
Beta-Ketothiolase DeficiencySMP00173Not Available
Isobutyryl-coa dehydrogenase deficiencySMP00523Not Available
Isovaleric acidemiaSMP00524Not Available
Isovaleric AciduriaSMP00238Not Available
Maple Syrup Urine DiseaseSMP00199Not Available
Methylmalonate Semialdehyde Dehydrogenase DeficiencySMP00384Not Available
Methylmalonic AciduriaSMP00200Not Available
Propionic AcidemiaSMP00236Not Available
Valine, Leucine and Isoleucine DegradationSMP00032map00280
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.03 +/- 0.34 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified7.0 +/- 6.6 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified10.0 +/- 9.0 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified4.3 +/- 2.9 umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022878
KNApSAcK IDNot Available
Chemspider ID388543
KEGG Compound IDC03284
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB02166
Metagene LinkHMDB02166
METLIN ID6520
PubChem Compound439434
PDB IDNot Available
ChEBI ID33094
References
Synthesis ReferenceAlauddin, Mian M.; Fissekis, John D.; Conti, Peter S. a-Alkylation of amino acid derivatives: synthesis and chiral resolution of [11C]b-aminoisobutyric acid. Nuclear Medicine and Biology (1997), 24(8), 771-775.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Van Kuilenburg AB, Stroomer AE, Van Lenthe H, Abeling NG, Van Gennip AH: New insights in dihydropyrimidine dehydrogenase deficiency: a pivotal role for beta-aminoisobutyric acid? Biochem J. 2004 Apr 1;379(Pt 1):119-24. [14705962 ]
  2. Roe CR, Struys E, Kok RM, Roe DS, Harris RA, Jakobs C: Methylmalonic semialdehyde dehydrogenase deficiency: psychomotor delay and methylmalonic aciduria without metabolic decompensation. Mol Genet Metab. 1998 Sep;65(1):35-43. [9787093 ]
  3. KAKIMOTO Y, KANAZAWA A, SANO I: IDENTIFICATION OF D(-)-BETA-AMINOISOBUTYRIC ACID IN HUMAN LIVER. Biochim Biophys Acta. 1965 Feb 15;97:376-7. [14292857 ]
  4. KAKIMOTO Y, ARMSTRONG MD: The preparation and isolation of D-(-)-beta-aminoisobutyric acid. J Biol Chem. 1961 Dec;236:3283-6. [14453202 ]

Enzymes

General function:
Involved in 4-aminobutyrate transaminase activity
Specific function:
Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine.
Gene Name:
ABAT
Uniprot ID:
P80404
Molecular weight:
56438.405
Reactions
(S)-b-aminoisobutyric acid + Oxoglutaric acid → 2-Methyl-3-oxopropanoic acid + L-Glutamic aciddetails
(S)-b-aminoisobutyric acid + Oxoglutaric acid → (S)-Methylmalonic acid semialdehyde + L-Glutamic aciddetails