(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
Structure for HMDB02166 ((S)-b-aminoisobutyric acid)
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
InChI Identifier
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
Substituents
Molecliar Framework
Aliphatic acyclic compounds External Descriptors
Ontology Status
Detected and Quantified Origin
Biofunction
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
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 Value Age Sex Condition Reference Details BloodDetected and Quantified1.03 +/- 0.34 uMAdlit (>18 years old)MaleNormaldetails FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details FecesDetected but not Quantified Adlit (>18 years old)Both
Normal
details UrineDetected and Quantified7.0 +/- 6.6 umol/mmol creatinineAdlit (>18 years old)FemaleNormal
details UrineDetected and Quantified10.0 +/- 9.0 umol/mmol creatinineAdlit (>18 years old)MaleNormal
details UrineDetected and Quantified4.3 +/- 2.9 umol/mmol creatinineChildren (1-13 years old)BothNormal
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
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