2.0 2012-05-31 13:55:58 -0600 2015-06-03 15:54:15 -0600 ECMDB02166 M2MDB000445 (S)-b-aminoisobutyric acid Beta-Aminoisobutyric acid is a non-protein amino acid originating from the catabolism of thymine and valine. Beta-Aminoisobutyric acid occurs in two isomeric forms. 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 abnormalities with a dihydropyrimidine dehydrogenase (DPD). 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 situation 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. (PMID: 14705962, 14292857, 14453202) (+)-a-Methyl-b-alanine (+)-alpha-Methyl-beta-alanine (+)-b-Aminoisobutyrate (+)-b-Aminoisobutyric acid (+)-beta-Aminoisobutyrate (+)-beta-Aminoisobutyric acid (+)-α-Methyl-β-alanine (+)-β-Aminoisobutyrate (+)-β-Aminoisobutyric acid (S)-3-amino-2-methyl-Propanoate (S)-3-amino-2-methyl-Propanoic acid (S)-3-Amino-2-methylpropanoate (S)-3-Amino-2-methylpropanoic acid (S)-3-Amino-isobutanoate (S)-3-Amino-isobutanoic acid (S)-3-Amino-isobutyrate (S)-3-Amino-isobutyric acid (S)-b-Aminoisobutyrate (S)-b-Aminoisobutyric acid (S)-beta-Aminoisobutyrate (S)-beta-Aminoisobutyric acid (S)-β-Aminoisobutyrate (S)-β-Aminoisobutyric acid L-2-Methyl-b-Alanine L-2-Methyl-beta-Alanine L-2-Methyl-β-alanine L-3-Amino-2-methylpropanoate L-3-Amino-2-methylpropanoic acid L-3-Amino-2-methylpropionate L-3-Amino-2-methylpropionic acid L-3-Amino-isobutanoate L-3-Amino-isobutanoic acid L-3-Amino-isobutyrate L-3-Amino-isobutyric acid L-b-Aminoisobutyrate L-b-Aminoisobutyric acid L-beta-Aminoisobutyrate L-beta-Aminoisobutyric acid L-β-Aminoisobutyrate L-β-Aminoisobutyric acid S-b-Aminoisobutyrate S-b-Aminoisobutyric acid S-beta-Aminoisobutyrate S-beta-Aminoisobutyric acid S-β-Aminoisobutyrate S-β-Aminoisobutyric acid C4H9NO2 103.1198 103.063328537 (2S)-3-amino-2-methylpropanoic acid L-β-aminoisobutyric acid 4249-19-8 C[C@@H](CN)C(O)=O InChI=1S/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)/t3-/m0/s1 QCHPKSFMDHPSNR-VKHMYHEASA-N Solid Cytoplasm Periplasm logp -2.97 ALOGPS logs 0.55 ALOGPS solubility 3.67e+02 g/l ALOGPS melting_point 175-177 oC logp -2.6 ChemAxon pka_strongest_acidic 4.17 ChemAxon pka_strongest_basic 10.32 ChemAxon iupac (2S)-3-amino-2-methylpropanoic acid ChemAxon average_mass 103.1198 ChemAxon mono_mass 103.063328537 ChemAxon smiles C[C@@H](CN)C(O)=O ChemAxon formula C4H9NO2 ChemAxon inchi InChI=1S/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)/t3-/m0/s1 ChemAxon inchikey QCHPKSFMDHPSNR-VKHMYHEASA-N ChemAxon polar_surface_area 63.32 ChemAxon refractivity 25.28 ChemAxon polarizability 10.42 ChemAxon rotatable_bond_count 2 ChemAxon acceptor_count 3 ChemAxon donor_count 2 ChemAxon physiological_charge 0 ChemAxon formal_charge 0 ChemAxon Valine, leucine and isoleucine degradation ec00280 Specdb::CMs 23210 Specdb::CMs 38294 Specdb::CMs 151967 Specdb::NmrOneD 149060 Specdb::NmrOneD 149061 Specdb::NmrOneD 149062 Specdb::NmrOneD 149063 Specdb::NmrOneD 149064 Specdb::NmrOneD 149065 Specdb::NmrOneD 149066 Specdb::NmrOneD 149067 Specdb::NmrOneD 149068 Specdb::NmrOneD 149069 Specdb::NmrOneD 149070 Specdb::NmrOneD 149071 Specdb::NmrOneD 149072 Specdb::NmrOneD 149073 Specdb::NmrOneD 149074 Specdb::NmrOneD 149075 Specdb::NmrOneD 149076 Specdb::NmrOneD 149077 Specdb::NmrOneD 149078 Specdb::NmrOneD 149079 Specdb::MsMs 25748 Specdb::MsMs 25749 Specdb::MsMs 25750 Specdb::MsMs 32306 Specdb::MsMs 32307 Specdb::MsMs 32308 Specdb::MsMs 1472777 Specdb::MsMs 1472778 Specdb::MsMs 1472779 Specdb::MsMs 1472780 Specdb::MsMs 1472781 Specdb::MsMs 1472782 Specdb::MsMs 1472783 Specdb::MsMs 1472784 Specdb::MsMs 1472785 Specdb::MsMs 1472786 Specdb::MsMs 1472787 Specdb::MsMs 1478290 Specdb::MsMs 1478291 Specdb::MsMs 1478292 Specdb::MsMs 1478293 Specdb::MsMs 1478294 Specdb::MsMs 1478295 Specdb::MsMs 1478296 Specdb::MsMs 1478297 HMDB02166 439434 388543 C03284 33094 BIB Kanehisa, M., Goto, S., Sato, Y., Furumichi, M., Tanabe, M. (2012). "KEGG for integration and interpretation of large-scale molecular data sets." Nucleic Acids Res 40:D109-D114. 22080510 Van Kuilenburg, A. B., Stroomer, A. E., Van Lenthe, H., Abeling, N. G., Van Gennip, A. H. (2004). "New insights in dihydropyrimidine dehydrogenase deficiency: a pivotal role for beta-aminoisobutyric acid?" Biochem J 379:119-124. 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. 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. 14292857 KAKIMOTO Y, ARMSTRONG MD: The preparation and isolation of D-(-)-beta-aminoisobutyric acid. J Biol Chem. 1961 Dec;236:3283-6. 14453202 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. 4-aminobutyrate aminotransferase P22256 GABT_ECOLI gabT http://ecmdb.ca/proteins/P22256.xml (S)-b-aminoisobutyric acid + alpha-Ketoglutarate <> (S)-Methylmalonic acid semialdehyde + L-Glutamate R04188 (S)-b-aminoisobutyric acid + Oxoglutaric acid > 2-Methyl-3-oxopropanoate + L-Glutamate