1.0 2011-09-21 00:26:35 UTC 2015-07-21 06:57:35 UTC FDB023226 2-Methylacetoacetic acid 2-Methylacetoacetic acid is a metabolite that has an increased excretion in patients with acetoacetyl-CoA thiolase (EC 2.3.1.9) deficiency (OMIM 607809, ACAT1; OMIM 100678, ACAT2; OMIM 604770, ACAA2; OMIM 607809). Thiolases are ubiquitous and important enzymes. Several isoenzymes are known, which can occur in the cytosol (ACAT2), the mitochondria (ACAT1) , or the peroxisomes(ACAA2). Thiolases are CoA-dependent enzymes which catalyze the formation of a carbon-carbon bond in a Claisen condensation step and its reverse reaction via a thiolytic degradation mechanism. mitochondrial acetoacetyl-coenzyme A (CoA) thiolase (T2) is important in the pathways for the synthesis and degradation of ketone bodies as well as for the degradation of 2-methylacetoacetyl-CoA. (PMID: 17371050, 14518824, 9744475, 9001814) [HMDB] (+/-)-2-Methyl-3-oxobutanoate (+/-)-2-Methyl-3-oxobutanoic acid 2-Acetylpropionic acid 2-Methyl-3-ketobutyric acid 2-methyl-3-oxo-Butanoate 2-methyl-3-oxo-Butanoic acid 2-Methyl-3-oxo-butyrate 2-Methyl-3-oxo-butyric acid 2-methyl-3-oxobutanoate 2-methyl-3-oxobutanoic acid 2-Methyl-3-oxobutyric acid 2-methyl-Acetoacetic acid 2-Methylacetoacetic acid 3-Oxo-2-methylbutyric acid a-Methylacetoacetic acid Alpha-methylacetoacetate alpha-Methylacetoacetic acid C5H8O3 116.1152 116.047344122 2-methyl-3-oxobutanoic acid acetopropionic acid 2382-59-4 CC(C(C)=O)C(O)=O InChI=1S/C5H8O3/c1-3(4(2)6)5(7)8/h3H,1-2H3,(H,7,8) GCXJINGJZAOJHR-UHFFFAOYSA-N belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms. Short-chain keto acids and derivatives Organic compounds Organic acids and derivatives Keto acids and derivatives Short-chain keto acids and derivatives Aliphatic acyclic compounds 1,3-dicarbonyl compounds Beta-hydroxy ketones Beta-keto acids and derivatives Carboxylic acids Hydrocarbon derivatives Methyl-branched fatty acids Monocarboxylic acids and derivatives Organic oxides 1,3-dicarbonyl compound Aliphatic acyclic compound Beta-hydroxy ketone Beta-keto acid Branched fatty acid Carbonyl group Carboxylic acid Carboxylic acid derivative Fatty acyl Hydrocarbon derivative Ketone Methyl-branched fatty acid Monocarboxylic acid or derivatives Organic oxide Organic oxygen compound Organooxygen compound Short-chain keto acid 3-oxo monocarboxylic acid Oxo fatty acids Solid logp 0.20 ALOGPS logs -0.09 ALOGPS solubility 9.49e+01 g/l ALOGPS logp 0.54 ChemAxon pka_strongest_acidic 4.17 ChemAxon pka_strongest_basic -7.5 ChemAxon iupac 2-methyl-3-oxobutanoic acid ChemAxon average_mass 116.1152 ChemAxon mono_mass 116.047344122 ChemAxon smiles CC(C(C)=O)C(O)=O ChemAxon formula C5H8O3 ChemAxon inchi InChI=1S/C5H8O3/c1-3(4(2)6)5(7)8/h3H,1-2H3,(H,7,8) ChemAxon inchikey GCXJINGJZAOJHR-UHFFFAOYSA-N ChemAxon polar_surface_area 54.37 ChemAxon refractivity 27.11 ChemAxon polarizability 11.09 ChemAxon rotatable_bond_count 2 ChemAxon acceptor_count 3 ChemAxon donor_count 1 ChemAxon physiological_charge -1 ChemAxon formal_charge 0 ChemAxon Specdb::CMs 25567 Specdb::CMs 38653 Specdb::MsMs 67791 Specdb::MsMs 67792 Specdb::MsMs 67793 Specdb::MsMs 125685 Specdb::MsMs 125686 Specdb::MsMs 125687 HMDB03771 37079 #<Reference:0x000055ce3331ce08> #<Reference:0x000055ce3331cc50> #<Reference:0x000055ce3331ca98> #<Reference:0x000055ce3331c8e0> Anatidae Type 1 specific Anatidae 8830 Beefalo Type 1 specific Bos taurus X Bison bison 297284 Bison Type 1 specific Bison bison 9901 Buffalo Type 1 specific Bubalus bubalis 89462 Cattle (Beef, Veal) Type 1 specific Bos taurus 9913 Chicken Type 1 specific Gallus gallus 9031 Columbidae (Dove, Pigeon) Type 1 specific Columbidae 8930 Deer Type 1 specific Cervidae 9850 Domestic goat Type 1 specific Capra aegagrus hircus 9925 Domestic pig Type 1 specific Sus scrofa domestica 9825 Elk Type 1 specific Cervus canadensis 1574408 Emu Type 1 specific Dromaius novaehollandiae 8790 European rabbit Type 1 specific Oryctolagus 9984 Greylag goose Type 1 specific Anser anser 8843 Guinea hen Type 1 specific Numida meleagris 8996 Horse Type 1 specific Equus caballus 9796 Mallard duck Type 1 specific Anas platyrhynchos 8839 Mountain hare Type 1 specific Lepus timidus 62621 Mule deer Type 1 specific Odocoileus 9871 Ostrich Type 1 specific Struthio camelus 8801 Pheasant Type 1 specific Phasianus colchicus 9054 Quail Type 1 specific Phasianidae 9005 Rabbit Type 1 specific Leporidae 9979 Rock ptarmigan Type 1 specific Lagopus muta 64668 Sheep (Mutton, Lamb) Type 1 specific Ovis aries 9940 Squab Type 1 specific Columba 8931 Turkey Type 1 specific Meleagris gallopavo 9103 Velvet duck Type 1 specific Melanitta fusca 371864 Wild boar Type 1 specific Sus scrofa 9823