2.02012-05-31 13:55:58 -06002015-06-03 15:54:15 -0600ECMDB02166M2MDB000445(S)-b-aminoisobutyric acidBeta-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 acidL-2-Methyl-b-AlanineL-2-Methyl-beta-AlanineL-2-Methyl-β-alanineL-3-Amino-2-methylpropanoateL-3-Amino-2-methylpropanoic acidL-3-Amino-2-methylpropionateL-3-Amino-2-methylpropionic acidL-3-Amino-isobutanoateL-3-Amino-isobutanoic acidL-3-Amino-isobutyrateL-3-Amino-isobutyric acidL-b-AminoisobutyrateL-b-Aminoisobutyric acidL-beta-AminoisobutyrateL-beta-Aminoisobutyric acidL-β-AminoisobutyrateL-β-Aminoisobutyric acidS-b-AminoisobutyrateS-b-Aminoisobutyric acidS-beta-AminoisobutyrateS-beta-Aminoisobutyric acidS-β-AminoisobutyrateS-β-Aminoisobutyric acidC4H9NO2103.1198103.063328537(2S)-3-amino-2-methylpropanoic acidL-β-aminoisobutyric acid4249-19-8C[C@@H](CN)C(O)=OInChI=1S/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)/t3-/m0/s1QCHPKSFMDHPSNR-VKHMYHEASA-NSolidCytoplasmPeriplasmlogp-2.97logs0.55solubility3.67e+02 g/lmelting_point175-177 oClogp-2.6pka_strongest_acidic4.17pka_strongest_basic10.32iupac(2S)-3-amino-2-methylpropanoic acidaverage_mass103.1198mono_mass103.063328537smilesC[C@@H](CN)C(O)=OformulaC4H9NO2inchiInChI=1S/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)/t3-/m0/s1inchikeyQCHPKSFMDHPSNR-VKHMYHEASA-Npolar_surface_area63.32refractivity25.28polarizability10.42rotatable_bond_count2acceptor_count3donor_count2physiological_charge0formal_charge0Valine, leucine and isoleucine degradationec00280Specdb::CMs23210Specdb::CMs38294Specdb::CMs151967Specdb::NmrOneD149060Specdb::NmrOneD149061Specdb::NmrOneD149062Specdb::NmrOneD149063Specdb::NmrOneD149064Specdb::NmrOneD149065Specdb::NmrOneD149066Specdb::NmrOneD149067Specdb::NmrOneD149068Specdb::NmrOneD149069Specdb::NmrOneD149070Specdb::NmrOneD149071Specdb::NmrOneD149072Specdb::NmrOneD149073Specdb::NmrOneD149074Specdb::NmrOneD149075Specdb::NmrOneD149076Specdb::NmrOneD149077Specdb::NmrOneD149078Specdb::NmrOneD149079Specdb::MsMs25748Specdb::MsMs25749Specdb::MsMs25750Specdb::MsMs32306Specdb::MsMs32307Specdb::MsMs32308Specdb::MsMs1472777Specdb::MsMs1472778Specdb::MsMs1472779Specdb::MsMs1472780Specdb::MsMs1472781Specdb::MsMs1472782Specdb::MsMs1472783Specdb::MsMs1472784Specdb::MsMs1472785Specdb::MsMs1472786Specdb::MsMs1472787Specdb::MsMs1478290Specdb::MsMs1478291Specdb::MsMs1478292Specdb::MsMs1478293Specdb::MsMs1478294Specdb::MsMs1478295Specdb::MsMs1478296Specdb::MsMs1478297HMDB02166439434388543C0328433094BIBKanehisa, 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-D114.22080510Van 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 379:119-124.14705962Roe 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):35-43.9787093KAKIMOTO Y, KANAZAWA A, SANO I: IDENTIFICATION OF D(-)-BETA-AMINOISOBUTYRIC ACID IN HUMAN LIVER. Biochim Biophys Acta. 1965 Feb 15;97:376-7.14292857KAKIMOTO Y, ARMSTRONG MD: The preparation and isolation of D-(-)-beta-aminoisobutyric acid. J Biol Chem. 1961 Dec;236:3283-6.14453202Alauddin, 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 aminotransferaseP22256GABT_ECOLIgabThttp://ecmdb.ca/proteins/P22256.xml(S)-b-aminoisobutyric acid + alpha-Ketoglutarate <> (S)-Methylmalonic acid semialdehyde + L-GlutamateR04188(S)-b-aminoisobutyric acid + Oxoglutaric acid > 2-Methyl-3-oxopropanoate + L-Glutamate