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Bovine Metabolome Database



Showing metabocard for (S)-b-aminoisobutyric acid (BMDB02166)

Legend: metabolite field enzyme field

Version 1.0
Creation Date 2006-05-22 15:17:39
Update Date 2009-05-05 20:59:08
Accession Number BMDB02166
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 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)
Synonyms
  1. L-b-Aminoisobutyric acid
  2. L-3-Amino-2-methylpropionic acid
  3. L-3-Amino-2-methylpropanoic acid
  4. L-2-methyl-b-Alanine
  5. (S)-b-Aminoisobutyric acid
  6. (S)-3-amino-2-methyl-Propanoic acid
  7. (+)-b-Aminoisobutyric acid
  8. (+)-a-Methyl-b-alanine
  9. S-b-aminoisobutyrate
  10. S-beta-aminoisobutyrate
  11. S-beta-aminoisobutyric acid
  12. L-beta-Aminoisobutyric acid
  13. L-2-methyl-beta-Alanine
  14. (S)-beta-Aminoisobutyric acid
  15. (+)-beta-Aminoisobutyric acid
  16. (+)-alpha-Methyl-beta-alanine
  17. L-beta-aminoisobutyrate
  18. L-b-aminoisobutyrate
  19. L-3-Amino-isobutanoate
  20. (S)-3-Amino-isobutyrate
  21. L-3-Amino-isobutyrate
  22. (S)-3-Amino-isobutanoate
  23. (S)-3-Amino-2-methylpropanoate
  24. L-3-Amino-isobutanoic acid
  25. (S)-3-Amino-isobutyric acid
  26. L-3-Amino-isobutyric acid
  27. (S)-3-Amino-isobutanoic acid
  28. (S)-3-Amino-2-methylpropanoic acid
  29. L-3-Amino-2-methylpropanoate
  30. (S)-3-amino-2-methyl-Propanoate
Chemical IUPAC Name (2S)-3-amino-2-methylpropanoic acid
Chemical Formula C4H9NO2
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Amino acids and Amino Acid conjugates
Class
  • Amino Acids
Sub Class
  • NA
Family
  • Mammalian_Metabolite
Species
  • primary amine; primary aliphatic amine (alkylamine); carboxylic acid
Biofunction
  • Protein synthesis, amino acid biosynthesis
Application
Source
  • Endogenous
Average Molecular Weight 103.120
Monoisotopic Molecular Weight 103.063332
Isomeric SMILES C[C@@H](CN)C(O)=O
Canonical SMILES CC(CN)C(O)=O
KEGG Compound ID C03284 Link Image
BioCyc ID Not Available
BiGG ID Not Available
Wikipedia Link Not Available
METLIN ID 6520 Link Image
PubChem Compound 439434 Link Image
PubChem Substance 15486482 Link Image
ChEBI ID 33094 Link Image
CAS Registry Number 4249-19-8
InChI Identifier InChI=1/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)/t3-/m0/s1
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.
Melting Point (Experimental) 175-177 oC
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 367.0 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge 0
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity -2.97 [Predicted by ALOGPS] Calculated using ALOGPS
Material Safety Data Sheet (MSDS) Not Available
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Not Available
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Not Available
Predicted 1H NMR Spectrum Show Image
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Predicted 13C NMR Spectrum Show Image
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Mass Spectrum Not Available
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location Not Available
Biofluid Location Not Available
Tissue Location Not Available
Concentrations (Normal) Not Available
Concentrations (Abnormal) Not Available
Pathway Names Not Available
HMDB Pathways Not Available
KEGG Pathways Not Available
SimCell Pathways Not Available
General References Not Available
Metabolic Enzymes
  1. 4-aminobutyrate aminotransferase, mitochondrial
Enzyme 1 [top]
Enzyme 1 ID 596
Enzyme 1 Name 4-aminobutyrate aminotransferase, mitochondrial
Enzyme 1 Synonyms
  1. Gamma-amino-N-butyrate transaminase
  2. GABA transaminase
  3. GABA-T
  4. GABA aminotransferase
  5. GABA-AT
  6. L-AIBAT
  7. (S)-3-amino-2-methylpropionate transaminase
Enzyme 1 Gene Name ABAT
Enzyme 1 Protein Sequence >4-aminobutyrate aminotransferase, mitochondrial
MASMLVAQRLACSFQHSYRLLVPGSRHISQAAAKVDVEFDYDGRLMKTEVPGLRCQELMK
QLNIIQNAEAVHFFCNYEESRGNYLVDVDGNRMLDLYSQISSVPIGYSHPGLLKLIQQPQ
NASMFVNRPALGILLPENFVEKLRQSLLSVAPKGMSQLITMACGSCSNENGLKTIFMWYR
SKERGQRGFPQEELETCMINQAPWCPDYSILSFMGAFHGRTMGCLATTHSKAIHKIDIPS
FDWPIAPFPRLKYPLEEFVKENQQEEARCLEEVEDLIVKYRKKKKTVAGIIVEPIQSEGG
DNHASDDFFRKLRDIPRKQCCAFLVDVVQTGGGCTGKFWAHEHWARDDPEDVMTSSKKMM
TGGFFHKEEFRPNAPYRIFNTWLGDPSKNLLLAEVINIIKREDLLNNAAHAGKALLTGLL
DLQARYPQFISRVRGRGTFCSFDTPDDSIRNKLILIARNKGVVLGGCGDKSIRFRPTLVF
RDHHAHLFLNIFSDILADFK
Enzyme 1 Number of Residues 500
Enzyme 1 Molecular Weight 56730.9
Enzyme 1 Theoretical pI 8.21
Enzyme 1 GO Classification
Function
Process
Component
Enzyme 1 General Function Amino acid transport and metabolism
Enzyme 1 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
Enzyme 1 Pathways Not Available
Enzyme 1 Reactions Not Available
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • None
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 12584862 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID Q9BGI0 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name GABT_BOVIN Link Image
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >1503 bp
ATGGCGTCCATGTTGGTCGCCCAGCGCCTGGCCTGCAGCTTCCAGCACAGCTACCGCCTG
CTGGTGCCTGGATCCAGACACATTAGTCAAGCTGCAGCCAAAGTCGACGTTGAATTTGAT
TATGATGGGCGTCTGATGAAGACGGAAGTCCCAGGGCTTAGATGTCAGGAGTTAATGAAA
CAGCTGAATATAATTCAGAATGCAGAGGCTGTGCATTTTTTCTGCAATTACGAAGAGAGC
CGAGGCAATTACCTGGTTGATGTGGACGGCAACCGAATGCTGGATCTTTATTCCCAGATC
TCCTCTGTCCCCATAGGTTACAGCCACCCCGGCCTGCTGAAACTCATCCAACAGCCTCAA
AATGCGAGCATGTTTGTCAACAGACCCGCCCTCGGAATCCTGCTTCCGGAGAACTTTGTG
GAGAAGCTCCGGCAGTCCTTGCTCTCGGTGGCTCCCAAAGGGATGTCCCAGCTCATCACC
ATGGCCTGCGGCTCCTGCTCCAATGAAAACGGCTTAAAGACCATCTTCATGTGGTACCGG
AGCAAGGAAAGAGGGCAGAGGGGCTTCCCCCAAGAGGAGCTGGAGACGTGCATGATTAAC
CAGGCCCCCTGGTGCCCCGACTACAGCATCCTCTCCTTCATGGGCGCGTTCCATGGGAGG
ACCATGGGTTGCTTAGCGACCACGCACTCTAAAGCCATTCACAAGATCGACATCCCTTCC
TTTGACTGGCCCATCGCACCGTTCCCACGGCTGAAATACCCTCTGGAAGAGTTTGTGAAA
GAGAACCAACAGGAGGAGGCCCGCTGTCTGGAAGAGGTGGAGGATCTGATTGTGAAATAT
CGGAAAAAGAAGAAGACGGTGGCCGGGATCATCGTGGAGCCCATCCAGTCCGAGGGTGGA
GACAACCACGCATCCGATGACTTCTTTCGGAAGCTGAGAGACATCCCCAGGAAGCAATGC
TGCGCCTTCTTGGTGGACGTGGTCCAGACCGGAGGAGGCTGCACGGGCAAGTTCTGGGCC
CACGAGCACTGGGCCCGGGATGACCCAGAAGACGTGATGACCTCCAGCAAGAAGATGATG
ACTGGGGGCTTCTTCCACAAGGAGGAGTTCAGGCCTAATGCTCCCTACCGGATCTTCAAC
ACCTGGCTGGGGGACCCGTCCAAGAACCTGTTGCTGGCTGAGGTCATCAACATCATCAAG
CGGGAGGACCTGCTAAATAATGCAGCCCATGCCGGGAAGGCCCTGCTCACAGGACTGCTG
GACCTCCAGGCCCGGTACCCCCAGTTCATCAGCAGGGTGAGAGGACGAGGCACCTTTTGC
TCCTTCGATACTCCCGATGATTCCATACGGAATAAGCTCATTTTAATTGCCAGAAACAAA
GGTGTGGTGTTGGGTGGCTGTGGTGACAAATCCATTCGTTTCCGTCCCACGCTGGTGTTC
AGGGATCACCACGCACACCTGTTCCTCAATATTTTCAGTGACATCTTAGCAGACTTCAAG
TAA
Enzyme 1 GenBank Gene ID AF305692 Link Image
Enzyme 1 GeneCard ID ABAT Link Image
Enzyme 1 GenAtlas ID Not Available
Enzyme 1 HGNC ID Not Available
Enzyme 1 Chromosome Location Chromosome:1
Enzyme 1 Locus 16p13.2
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available