1.0 2016-09-30 22:26:39 UTC 2020-06-04 19:31:06 UTC BMDB0000202 BMDB00202 Methylmalonic acid Methylmalonic acid, also known as 2-methylmalonate or methyl-propanedioate, belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups. Methylmalonic acid exists as a solid, very hydrophobic, practically insoluble (in water), and relatively neutral molecule. Methylmalonic acid exists in all living species, ranging from bacteria to humans. In cattle, methylmalonic acid is involved in the metabolic pathway called the vitamin K metabolism pathway. Methylmalonic acid is a potentially toxic compound. Methylmalonic acid has been found to be associated with several diseases known as eosinophilic esophagitis, combined malonic and methylmalonic aciduria, alzheimer's disease, and methylmalonic aciduria mitochondrial encephelopathy leigh-like; also methylmalonic acid has been linked to the inborn metabolic disorders including vitamin B12 deficiency. 1,1-Ethanedicarboxylic acid 2-Methylmalonic acid alpha-Methylmalonic acid Isosuccinic acid 1,1-Ethanedicarboxylate 2-Methylmalonate a-Methylmalonate a-Methylmalonic acid alpha-Methylmalonate Α-methylmalonate Α-methylmalonic acid Isosuccinate Methylmalonate Methyl-malonate Methyl-malonic acid Methyl-propanedioate Methyl-propanedioic acid Methylpropanedioate Methylpropanedioic acid Acid, methylmalonic Methylmalonic acid C4H6O4 118.088 118.02660868 2-methylpropanedioic acid methylmalonic acid 516-05-2 CC(C(O)=O)C(O)=O InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8) ZIYVHBGGAOATLY-UHFFFAOYSA-N belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups. Organic compounds Organic acids and derivatives Carboxylic acids and derivatives Dicarboxylic acids and derivatives Dicarboxylic acids and derivatives 1,3-dicarbonyl compounds Carboxylic acids Hydrocarbon derivatives Organic oxides 1,3-dicarbonyl compound Aliphatic acyclic compound Carbonyl group Carboxylic acid Dicarboxylic acid or derivatives Hydrocarbon derivative Organic oxide Organic oxygen compound Organooxygen compound Aliphatic acyclic compounds C4-dicarboxylic acid Dicarboxylic acids Solid melting_point 135 °C water_solubility 679.0 mg/mL logp 0.17 ALOGPS logs 0.10 ALOGPS logp 0.21 ChemAxon pka_strongest_acidic 2.48 ChemAxon iupac 2-methylpropanedioic acid ChemAxon average_mass 118.088 ChemAxon mono_mass 118.02660868 ChemAxon smiles CC(C(O)=O)C(O)=O ChemAxon formula C4H6O4 ChemAxon inchi InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8) ChemAxon inchikey ZIYVHBGGAOATLY-UHFFFAOYSA-N ChemAxon polar_surface_area 74.6 ChemAxon refractivity 23.56 ChemAxon polarizability 10.06 ChemAxon rotatable_bond_count 2 ChemAxon acceptor_count 4 ChemAxon donor_count 2 ChemAxon physiological_charge -2 ChemAxon formal_charge 0 ChemAxon number_of_rings 0 ChemAxon bioavailability 1 ChemAxon rule_of_five Yes ChemAxon ghose_filter Yes ChemAxon veber_rule Yes ChemAxon mddr_like_rule Yes ChemAxon Propanoate Metabolism SMP0087248 Valine, Leucine, and Isoleucine Degradation SMP0087234 Vitamin K Metabolism SMP0087227 Specdb::MsMs 325 Specdb::MsMs 326 Specdb::MsMs 327 Specdb::MsMs 3590 Specdb::MsMs 3591 Specdb::MsMs 3592 Specdb::MsMs 3593 Specdb::MsMs 3594 Specdb::MsMs 180024 Specdb::MsMs 180025 Specdb::MsMs 180026 Specdb::MsMs 182358 Specdb::MsMs 182359 Specdb::MsMs 182360 Specdb::MsMs 438075 Specdb::MsMs 438076 Specdb::MsMs 438077 Specdb::MsMs 438078 Specdb::MsMs 438079 Specdb::MsMs 2235969 Specdb::MsMs 2236625 Specdb::MsMs 2236726 Specdb::MsMs 2238140 Specdb::MsMs 2238778 Specdb::MsMs 2238826 Specdb::NmrTwoD 1203 Specdb::NmrOneD 1183 Specdb::NmrOneD 1201 Specdb::NmrOneD 2429 Specdb::NmrOneD 3116 Specdb::NmrOneD 5057 Specdb::NmrOneD 5058 Specdb::NmrOneD 166478 Specdb::EiMs 476 Specdb::MsIr 287 Specdb::MsIr 288 Specdb::MsIr 289 Specdb::CMs 456 Specdb::CMs 457 Specdb::CMs 969 Specdb::CMs 1101 Specdb::CMs 3366 Specdb::CMs 29830 Specdb::CMs 30218 Specdb::CMs 30834 Specdb::CMs 31066 Specdb::CMs 31067 Specdb::CMs 31868 Specdb::CMs 37353 Specdb::CMs 156464 Specdb::CMs 1054021 Specdb::CMs 1054023 Specdb::CMs 1054024 Kidney Wishart DS, Feunang YD, Marcu A, Guo AC, Liang K, Vazquez-Fresno R, Sajed T, Johnson D, Li C, Karu N, Sayeeda Z, Lo E, Assempour N, Berjanskii M, Singhal S, Arndt D, Liang Y, Badran H, Grant J, Serra-Cayuela A, Liu Y, Mandal R, Neveu V, Pon A, Knox C, Wilson M, Manach C, Scalbert A: HMDB 4.0: the human metabolome database for 2018. Nucleic Acids Res. 2018 Jan 4;46(D1):D608-D617. doi: 10.1093/nar/gkx1089. 29140435 Liver 8 multiparous Chinese Holstein dairy cows fed in the Hangzhou Hangjiang Dairy Farm based on the milk production under corn stover based diets. Detection used gas chromatography time-of-flight/mass spectrometry (GC-TOF/MS) platform. Sun HZ, Zhou M, Wang O, Chen Y, Liu JX, Guan LL: Multi-omics reveals functional genomic and metabolic mechanisms of milk production and quality in dairy cows. Bioinformatics. 2020 Apr 15;36(8):2530-2537. doi: 10.1093/bioinformatics/btz951. 31873721 Liver 8 multiparous Chinese Holstein dairy cows fed in the Hangzhou Hangjiang Dairy Farm based on the milk production under alfalfa hay based diets. Detection used gas chromatography time-of-flight/mass spectrometry (GC-TOF/MS) platform. Sun HZ, Zhou M, Wang O, Chen Y, Liu JX, Guan LL: Multi-omics reveals functional genomic and metabolic mechanisms of milk production and quality in dairy cows. Bioinformatics. 2020 Apr 15;36(8):2530-2537. doi: 10.1093/bioinformatics/btz951. 31873721 Mammary Gland 8 multiparous Chinese Holstein dairy cows fed in the Hangzhou Hangjiang Dairy Farm based on the milk production under corn stover based diets. Detection used gas chromatography time-of-flight/mass spectrometry (GC-TOF/MS) platform. Sun HZ, Zhou M, Wang O, Chen Y, Liu JX, Guan LL: Multi-omics reveals functional genomic and metabolic mechanisms of milk production and quality in dairy cows. Bioinformatics. 2020 Apr 15;36(8):2530-2537. doi: 10.1093/bioinformatics/btz951. 31873721 Mammary Gland 8 multiparous Chinese Holstein dairy cows fed in the Hangzhou Hangjiang Dairy Farm based on the milk production under alfalfa hay based diets. Detection used gas chromatography time-of-flight/mass spectrometry (GC-TOF/MS) platform. Sun HZ, Zhou M, Wang O, Chen Y, Liu JX, Guan LL: Multi-omics reveals functional genomic and metabolic mechanisms of milk production and quality in dairy cows. Bioinformatics. 2020 Apr 15;36(8):2530-2537. doi: 10.1093/bioinformatics/btz951. 31873721 Milk Raw milk, by NMR and GC-MS Antunes-Fernandes EC, van Gastelen S, Dijkstra J, Hettinga KA, Vervoort J: Milk metabolome relates enteric methane emission to milk synthesis and energy metabolism pathways. J Dairy Sci. 2016 Aug;99(8):6251-6262. doi: 10.3168/jds.2015-10248. Epub 2016 May 26. 27236769 Placenta Wishart DS, Feunang YD, Marcu A, Guo AC, Liang K, Vazquez-Fresno R, Sajed T, Johnson D, Li C, Karu N, Sayeeda Z, Lo E, Assempour N, Berjanskii M, Singhal S, Arndt D, Liang Y, Badran H, Grant J, Serra-Cayuela A, Liu Y, Mandal R, Neveu V, Pon A, Knox C, Wilson M, Manach C, Scalbert A: HMDB 4.0: the human metabolome database for 2018. Nucleic Acids Res. 2018 Jan 4;46(D1):D608-D617. doi: 10.1093/nar/gkx1089. 29140435 Ruminal Fluid 0.593 +/- 0.043 uM Girard CL, Matte JJ: Effects of intramuscular injections of vitamin B12 on lactation performance of dairy cows fed dietary supplements of folic acid and rumen-protected methionine. J Dairy Sci. 2005 Feb;88(2):671-6. doi: 10.3168/jds.S0022-0302(05)72731-4. 15653534 C02170 FDB021905 DB04183 473 30860 487 CPD-546 Methylmalonic_acid 3712 Edamura, Koji; Arai, Takeshi. Malonic and methylmalonic acids from b-hydroxypropionic acids. Jpn. Kokai Tokkyo Koho (1979), 5 pp. Sun HZ, Shi K, Wu XH, Xue MY, Wei ZH, Liu JX, Liu HY: Lactation-related metabolic mechanism investigated based on mammary gland metabolomics and 4 biofluids' metabolomics relationships in dairy cows. BMC Genomics. 2017 Dec 2;18(1):936. doi: 10.1186/s12864-017-4314-1. 29197344 Antunes-Fernandes EC, van Gastelen S, Dijkstra J, Hettinga KA, Vervoort J: Milk metabolome relates enteric methane emission to milk synthesis and energy metabolism pathways. J Dairy Sci. 2016 Aug;99(8):6251-6262. doi: 10.3168/jds.2015-10248. Epub 2016 May 26. 27236769 BMDBP00677 Aldehyde oxidase 1 P48034 AOX1 Enzyme BMDBP00990 Aldehyde dehydrogenase, mitochondrial P20000 ALDH2 Enzyme