2.0 2012-05-31 13:56:08 -0600 2015-06-03 15:54:15 -0600 ECMDB02217 M2MDB000448 (S)-Methylmalonic acid semialdehyde Methylmalonic semialdehyde is a metabolite in valine catabolism, inositol metabolism and propanoate metabolism. Methylmalonate-semialdehyde dehydrogenase (MMSDH) catalyses the NAD+ and coenzyme A-dependent conversion of methylmalonate semialdehyde to propionyl-CoA in the distal region of the L-valine catabolic pathway. Direct enzymatic assay of MMSDH is difficult since the substrate, methylmalonate semialdehyde, is both commercially unavailable and notoriously unstable as a b-keto acid. (PMID: 10947204) (2S)-2-methyl-3-oxopropanoate (2S)-2-methyl-3-oxopropanoic acid (S)-Methylmalonate semialdehyde C4H6O3 102.0886 102.031694058 (2S)-2-methyl-3-oxopropanoic acid (S)-methylmalonaldehydic acid 99043-16-0 C[C@@H](C=O)C(O)=O InChI=1S/C4H6O3/c1-3(2-5)4(6)7/h2-3H,1H3,(H,6,7)/t3-/m0/s1 VOKUMXABRRXHAR-VKHMYHEASA-N Solid Outer membrane Inner membrane logp -0.00 ALOGPS logs 0.42 ALOGPS solubility 2.66e+02 g/l ALOGPS logp 0.053 ChemAxon pka_strongest_acidic 3.98 ChemAxon pka_strongest_basic -7.2 ChemAxon iupac (2S)-2-methyl-3-oxopropanoic acid ChemAxon average_mass 102.0886 ChemAxon mono_mass 102.031694058 ChemAxon smiles C[C@@H](C=O)C(O)=O ChemAxon formula C4H6O3 ChemAxon inchi InChI=1S/C4H6O3/c1-3(2-5)4(6)7/h2-3H,1H3,(H,6,7)/t3-/m0/s1 ChemAxon inchikey VOKUMXABRRXHAR-VKHMYHEASA-N ChemAxon polar_surface_area 54.37 ChemAxon refractivity 22.64 ChemAxon polarizability 9.16 ChemAxon rotatable_bond_count 2 ChemAxon acceptor_count 3 ChemAxon donor_count 1 ChemAxon physiological_charge -1 ChemAxon formal_charge 0 ChemAxon Propanoate metabolism Starting from L-threonine, this compound is deaminated through a threonine deaminase resulting in a hydrogen ion, a water molecule and a (2z)-2-aminobut-2-enoate. The latter compound then isomerizes to a 2-iminobutanoate, This compound then reacts spontaneously with hydrogen ion and a water molecule resulting in a ammonium and a 2-Ketobutyric acid. The latter compound interacts with CoA through a pyruvate formate-lyase / 2-ketobutyrate formate-lyase resulting in a formic acid and a propionyl-CoA. Propionyl-CoA can then be processed either into a 2-methylcitric acid or into a propanoyl phosphate. Propionyl-CoA interacts with oxalacetic acid and a water molecule through a 2-methylcitrate synthase resulting in a hydrogen ion, a CoA and a 2-Methylcitric acid.The latter compound is dehydrated through a 2-methylcitrate dehydratase resulting in a water molecule and cis-2-methylaconitate. The latter compound is then dehydrated by a bifunctional aconitate hydratase 2 and 2-methylisocitrate dehydratase resulting in a water molecule and methylisocitric acid. The latter compound is then processed by 2-methylisocitrate lyase resulting in a release of succinic acid and pyruvic acid. Succinic acid can then interact with a propionyl-CoA through a propionyl-CoA:succinate CoA transferase resulting in a propionic acid and a succinyl CoA. Succinyl-CoA is then isomerized through a methylmalonyl-CoA mutase resulting in a methylmalonyl-CoA. This compound is then decarboxylated through a methylmalonyl-CoA decarboxylase resulting in a release of Carbon dioxide and Propionyl-CoA. ropionyl-CoA interacts with a phosphate through a phosphate acetyltransferase / phosphate propionyltransferase resulting in a CoA and a propanoyl phosphate. Propionyl-CoA can react with a phosphate through a phosphate acetyltransferase / phosphate propionyltransferase resulting in a CoA and a propanoyl phosphate. The latter compound is then dephosphorylated through a ADP driven acetate kinase/propionate kinase protein complex resulting in an ATP and Propionic acid. Propionic acid can be processed by a reaction with CoA through a ATP-driven propionyl-CoA synthetase resulting in a pyrophosphate, an AMP and a propionyl-CoA. PW000940 ec00640 Metabolic Valine, leucine and isoleucine degradation ec00280 Trinitrotoluene degradation ec00633 Microbial metabolism in diverse environments ec01120 Specdb::CMs 18408 Specdb::CMs 38322 Specdb::CMs 173491 Specdb::NmrOneD 332718 Specdb::NmrOneD 332719 Specdb::NmrOneD 332720 Specdb::NmrOneD 332721 Specdb::NmrOneD 332722 Specdb::NmrOneD 332723 Specdb::NmrOneD 332724 Specdb::NmrOneD 332725 Specdb::NmrOneD 332726 Specdb::NmrOneD 332727 Specdb::NmrOneD 332728 Specdb::NmrOneD 332729 Specdb::NmrOneD 332730 Specdb::NmrOneD 332731 Specdb::NmrOneD 332732 Specdb::NmrOneD 332733 Specdb::NmrOneD 332734 Specdb::NmrOneD 332735 Specdb::NmrOneD 332736 Specdb::NmrOneD 332737 Specdb::MsMs 28961 Specdb::MsMs 28962 Specdb::MsMs 28963 Specdb::MsMs 35519 Specdb::MsMs 35520 Specdb::MsMs 35521 Specdb::MsMs 2455066 Specdb::MsMs 2455067 Specdb::MsMs 2455068 Specdb::MsMs 2479726 Specdb::MsMs 2479727 Specdb::MsMs 2479728 HMDB02217 5462303 4575365 C06002 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 Chambliss, K. L., Gray, R. G., Rylance, G., Pollitt, R. J., Gibson, K. M. (2000). "Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency." J Inherit Metab Dis 23:497-504. 10947204 Manning NJ, Pollitt RJ: Tracer studies of the interconversion of R- and S-methylmalonic semialdehydes in man. Biochem J. 1985 Oct 15;231(2):481-4. 4062908 Fatty acid oxidation complex subunit alpha P21177 FADB_ECOLI fadB http://ecmdb.ca/proteins/P21177.xml 4-aminobutyrate aminotransferase P22256 GABT_ECOLI gabT http://ecmdb.ca/proteins/P22256.xml Fatty acid oxidation complex subunit alpha_ P77399 FADJ_ECOLI fadJ http://ecmdb.ca/proteins/P77399.xml Fatty acid oxidation complex subunit alpha P21177 FADB_ECOLI fadB http://ecmdb.ca/proteins/P21177.xml (S)-b-aminoisobutyric acid + alpha-Ketoglutarate <> (S)-Methylmalonic acid semialdehyde + L-Glutamate R04188 (S)-3-Hydroxyisobutyrate + NAD <> (S)-Methylmalonic acid semialdehyde + NADH + Hydrogen ion R05066