| Common Name |
(S)-b-aminoisobutyric acid
| Description |
Beta-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 tubliar 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 accumliation of S-beta-Aminoisobutyric acid in plasma. (PMID: 14705962 , 14292857 , 14453202 ).
| Structure |
MOLSDF3D-SDFPDBSMILESInChI View 3D Structure
| Synonyms |
| Value |
Source |
(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)-b-AminoisobutyrateGenerator
(S)-3-amino-IsobutanoateGenerator
(S)-3-amino-IsobutyrateGenerator
(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 Formlia |
C4H9NO2
| Average Molecliar Weight |
103.1198
| Monoisotopic Molecliar Weight |
103.063328537
| IUPAC Name |
(2S)-3-amino-2-methylpropanoic acid
| Traditional Name |
(+)-α-methyl-β-alanine
| CAS Registry Number |
4249-19-8
| 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 Key |
QCHPKSFMDHPSNR-VKHMYHEASA-N
| Chemical Taxonomy |
| Description |
This compound belongs to the class of chemical entities known as beta amino acids and derivatives. These are amino acids having a (-NH2) group attached to the beta carbon atom.
| Kingdom |
Chemical entities
| Super Class |
Organic compounds
| Class |
Organic acids and derivatives
| Sub Class |
Carboxylic acids and derivatives
| Direct Parent |
Beta amino acids and derivatives
| Alternative Parents |
Amino acids
Monocarboxylic acids and derivatives
Carboxylic acids
Organopnictogen compounds
Organic oxides
Monoalkylamines
Hydrocarbon derivatives
Carbonyl compounds
| Substituents |
Beta amino acid or derivatives
Amino acid
Carboxylic acid
Monocarboxylic acid or derivatives
Amine
Organic oxide
Hydrocarbon derivative
Primary amine
Organooxygen compound
Organonitrogen compound
Organopnictogen compound
Primary aliphatic amine
Organic oxygen compound
Carbonyl group
Organic nitrogen compound
Aliphatic acyclic compound
| Molecliar Framework |
Aliphatic acyclic compounds
| External Descriptors |
beta-amino acid (CHEBI:33094 )
3-aminoisobutyric acid (CHEBI:33094 )
Amino fatty acids (LMFA01100050 )
| Ontology |
| Status |
Detected and Quantified
| Origin |
Endogenous
| Biofunction |
Protein synthesis, amino acid biosynthesis
| Application |
Not Available
| Cellliar locations |
Not Available
| Physical Properties |
| State |
Solid
| Experimental Properties |
| Property |
Value |
Reference |
Melting Point175 – 177 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
| Predicted Properties |
| Property |
Value |
Source |
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
Rlie of FiveYesChemAxon
Ghose FilterYesChemAxon
Vebers RlieYesChemAxon
MDDR-like RlieYesChemAxon
| Spectra |
| Spectra |
| Spectrum Type |
Description |
Splash Key |
|
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 10V, Positivesplash10-0f79-9300000000-30669ed04aadf3d3f040View in MoNA
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 20V, Positivesplash10-000l-9000000000-ca65637476e64152c44eView in MoNA
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 40V, Positivesplash10-0006-9000000000-04d1648391d3f90db226View in MoNA
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 10V, Negativesplash10-0udi-3900000000-ec54fcce079024a62344View in MoNA
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 20V, Negativesplash10-0zfr-9500000000-0394f399cdf5df93c5dfView in MoNA
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 40V, Negativesplash10-0a4l-9000000000-d0c495c908ed905558a2View in MoNA
| Biological Properties |
| Cellliar Locations |
Not Available
| Biofluid Locations |
Blood
Feces
Urine
| Tissue Location |
Not Available
| Pathways |
| Name |
SMPDB Link |
KEGG 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 |
| Biofluid |
Status |
Age |
Condition |
Reference |
Details |
BloodDetected and Quantified1.03 +/- 0.34 uMAdlit (>18 years old)MaleNormal
Geigy Scientific …
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
20669995
details
FecesDetected but not Quantified Adlit (>18 years old)Both
Normal
24029555
details
UrineDetected and Quantified7.0 +/- 6.6 umol/mmol creatinineAdlit (>18 years old)FemaleNormal
Geigy Scientific …
details
UrineDetected and Quantified10.0 +/- 9.0 umol/mmol creatinineAdlit (>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 References |
None
| Associated OMIM IDs |
None
| External Links |
| DrugBank ID |
Not Available
| DrugBank Metabolite ID |
Not Available
| Phenol Explorer Compound ID |
Not Available
| Phenol Explorer Metabolite ID |
Not Available
| FoodDB ID |
FDB022878
| KNApSAcK ID |
Not Available
| Chemspider ID |
388543
| KEGG Compound ID |
C03284
| BioCyc ID |
Not Available
| BiGG ID |
Not Available
| Wikipedia Link |
Not Available
| NuGOwiki Link |
HMDB02166
| Metagene Link |
HMDB02166
| METLIN ID |
6520
| PubChem Compound |
439434
| PDB ID |
Not Available
| ChEBI ID |
33094
Product: 4-Aminoantipyrine
References |
| Synthesis Reference |
Alauddin, 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 |
- 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. [PubMed:14705962 ]
- 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. [PubMed:9787093 ]
- KAKIMOTO Y, KANAZAWA A, SANO I: IDENTIFICATION OF D(-)-BETA-AMINOISOBUTYRIC ACID IN HUMAN LIVER. Biochim Biophys Acta. 1965 Feb 15;97:376-7. [PubMed:14292857 ]
- KAKIMOTO Y, ARMSTRONG MD: The preparation and isolation of D-(-)-beta-aminoisobutyric acid. J Biol Chem. 1961 Dec;236:3283-6. [PubMed: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 acid |
details |
| (S)-b-aminoisobutyric acid + Oxoglutaric acid → (S)-Methylmalonic acid semialdehyde + L-Glutamic acid |
details |
PMID: 23152849
