1.0 2011-09-21 00:21:59 UTC 2015-07-21 06:57:25 UTC FDB022936 (E)-2-Methylglutaconic acid 2-Methylglutaconic acid is found in the urine of patients with organic aciduria from 2-methylacetoacetyl-CoA thiolase (EC 2.3.1.9, acetyl-CoA C-acetyltransferase) deficiency (ACAT, OMIM 607809). Main clinical features of ACAT include important staturo-ponderal delay, frequent infectious rhinopharyngitis episodes and an acute metabolic acidosis (this metabolic decompensation being adequately halted by bicarbonate supplementation). (PMID: 8930414, 2925825) [HMDB] (E)-2-methyl-2-Pentenedioate (E)-2-methyl-2-Pentenedioic acid (E)-2-Methylglutaconate trans-2-Methylglutaconate trans-2-Methylglutaconic acid C6H8O4 144.1253 144.042258744 (2E)-2-methylpent-2-enedioic acid 2E-methyl-glutaconic acid 53358-21-7 C\C(=C/CC(O)=O)C(O)=O InChI=1S/C6H8O4/c1-4(6(9)10)2-3-5(7)8/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+ JKGHDBJDBRBRNA-DUXPYHPUSA-N belongs to the class of organic compounds known as methyl-branched fatty acids. These are fatty acids with an acyl chain that has a methyl branch. Usually, they are saturated and contain only one or more methyl group. However, branches other than methyl may be present. Methyl-branched fatty acids Organic compounds Lipids and lipid-like molecules Fatty Acyls Fatty acids and conjugates Aliphatic acyclic compounds Carbonyl compounds Carboxylic acids Dicarboxylic acids and derivatives Hydrocarbon derivatives Organic oxides Unsaturated fatty acids Aliphatic acyclic compound Carbonyl group Carboxylic acid Carboxylic acid derivative Dicarboxylic acid or derivatives Hydrocarbon derivative Methyl-branched fatty acid Organic oxide Organic oxygen compound Organooxygen compound Unsaturated fatty acid Dicarboxylic acids Solid logp 0.30 ALOGPS logs -1.22 ALOGPS solubility 8.75e+00 g/l ALOGPS logp 0.44 ChemAxon pka_strongest_acidic 3.84 ChemAxon iupac (2E)-2-methylpent-2-enedioic acid ChemAxon average_mass 144.1253 ChemAxon mono_mass 144.042258744 ChemAxon smiles C\C(=C/CC(O)=O)C(O)=O ChemAxon formula C6H8O4 ChemAxon inchi InChI=1S/C6H8O4/c1-4(6(9)10)2-3-5(7)8/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+ ChemAxon inchikey JKGHDBJDBRBRNA-DUXPYHPUSA-N ChemAxon polar_surface_area 74.6 ChemAxon refractivity 33.59 ChemAxon polarizability 13.13 ChemAxon rotatable_bond_count 3 ChemAxon acceptor_count 4 ChemAxon donor_count 2 ChemAxon physiological_charge -2 ChemAxon formal_charge 0 ChemAxon Specdb::MsIr 7 Specdb::MsIr 1713 Specdb::MsIr 1714 Specdb::MsIr 1715 Specdb::CMs 12035 Specdb::CMs 38345 Specdb::CMs 152765 Specdb::NmrOneD 115798 Specdb::NmrOneD 115799 Specdb::NmrOneD 115800 Specdb::NmrOneD 115801 Specdb::NmrOneD 115802 Specdb::NmrOneD 115803 Specdb::NmrOneD 115804 Specdb::NmrOneD 115805 Specdb::NmrOneD 115806 Specdb::NmrOneD 115807 Specdb::NmrOneD 115808 Specdb::NmrOneD 115809 Specdb::NmrOneD 115810 Specdb::NmrOneD 115811 Specdb::NmrOneD 115812 Specdb::NmrOneD 115813 Specdb::NmrOneD 115814 Specdb::NmrOneD 115815 Specdb::NmrOneD 115816 Specdb::NmrOneD 115817 Specdb::MsMs 94794 Specdb::MsMs 94795 Specdb::MsMs 94796 Specdb::MsMs 158910 Specdb::MsMs 158911 Specdb::MsMs 158912 Specdb::MsMs 2328699 Specdb::MsMs 2328700 Specdb::MsMs 2328701 Specdb::MsMs 2633930 Specdb::MsMs 2633931 Specdb::MsMs 2633932 HMDB02266 #<Reference:0x000055ce31f66198> #<Reference:0x000055ce31f65fe0> #<Reference:0x000055ce31f65e28> 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 Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial Q9HCC0 MCCC2 Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial Q96RQ3 MCCC1