1.02011-09-21 00:26:35 UTC2015-07-21 06:57:35 UTCFDB0232262-Methylacetoacetic acid2-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 acid2-Acetylpropionic acid2-Methyl-3-ketobutyric acid2-methyl-3-oxo-Butanoate2-methyl-3-oxo-Butanoic acid2-Methyl-3-oxo-butyrate2-Methyl-3-oxo-butyric acid2-methyl-3-oxobutanoate2-methyl-3-oxobutanoic acid2-Methyl-3-oxobutyric acid2-methyl-Acetoacetic acid2-Methylacetoacetic acid3-Oxo-2-methylbutyric acida-Methylacetoacetic acidAlpha-methylacetoacetatealpha-Methylacetoacetic acidC5H8O3116.1152116.0473441222-methyl-3-oxobutanoic acidacetopropionic acid2382-59-4CC(C(C)=O)C(O)=OInChI=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 derivativesOrganic compoundsOrganic acids and derivativesKeto acids and derivativesShort-chain keto acids and derivativesAliphatic acyclic compounds1,3-dicarbonyl compoundsBeta-hydroxy ketonesBeta-keto acids and derivativesCarboxylic acidsHydrocarbon derivativesMethyl-branched fatty acidsMonocarboxylic acids and derivativesOrganic oxides1,3-dicarbonyl compoundAliphatic acyclic compoundBeta-hydroxy ketoneBeta-keto acidBranched fatty acidCarbonyl groupCarboxylic acidCarboxylic acid derivativeFatty acylHydrocarbon derivativeKetoneMethyl-branched fatty acidMonocarboxylic acid or derivativesOrganic oxideOrganic oxygen compoundOrganooxygen compoundShort-chain keto acid3-oxo monocarboxylic acidOxo fatty acidsSolidlogp0.20logs-0.09solubility9.49e+01 g/llogp0.54pka_strongest_acidic4.17pka_strongest_basic-7.5iupac2-methyl-3-oxobutanoic acidaverage_mass116.1152mono_mass116.047344122smilesCC(C(C)=O)C(O)=OformulaC5H8O3inchiInChI=1S/C5H8O3/c1-3(4(2)6)5(7)8/h3H,1-2H3,(H,7,8)inchikeyGCXJINGJZAOJHR-UHFFFAOYSA-Npolar_surface_area54.37refractivity27.11polarizability11.09rotatable_bond_count2acceptor_count3donor_count1physiological_charge-1formal_charge0Specdb::CMs25567Specdb::CMs38653Specdb::MsMs67791Specdb::MsMs67792Specdb::MsMs67793Specdb::MsMs125685Specdb::MsMs125686Specdb::MsMs125687HMDB0377137079#<Reference:0x0000555676255130>#<Reference:0x0000555676254f78>#<Reference:0x0000555676254dc0>#<Reference:0x0000555676254c08>AnatidaeType 1specificAnatidae8830BeefaloType 1specificBos taurus X Bison bison297284BisonType 1specificBison bison9901BuffaloType 1specificBubalus bubalis89462Cattle (Beef, Veal)Type 1specificBos taurus9913ChickenType 1specificGallus gallus9031Columbidae (Dove, Pigeon)Type 1specificColumbidae8930DeerType 1specificCervidae9850Domestic goatType 1specificCapra aegagrus hircus9925Domestic pigType 1specificSus scrofa domestica9825ElkType 1specificCervus canadensis1574408EmuType 1specificDromaius novaehollandiae8790European rabbitType 1specificOryctolagus9984Greylag gooseType 1specificAnser anser8843Guinea henType 1specificNumida meleagris8996HorseType 1specificEquus caballus9796Mallard duckType 1specificAnas platyrhynchos8839Mountain hareType 1specificLepus timidus62621Mule deerType 1specificOdocoileus9871OstrichType 1specificStruthio camelus8801PheasantType 1specificPhasianus colchicus9054QuailType 1specificPhasianidae9005RabbitType 1specificLeporidae9979Rock ptarmiganType 1specificLagopus muta64668Sheep (Mutton, Lamb)Type 1specificOvis aries9940SquabType 1specificColumba8931TurkeyType 1specificMeleagris gallopavo9103Velvet duckType 1specificMelanitta fusca371864Wild boarType 1specificSus scrofa9823