1.0 2011-09-21 00:05:57 UTC 2015-10-09 22:33:40 UTC FDB021963 2-Methylbutyrylglycine 2-Methylbutyrylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) which is an enzyme that catalyzes the chemical reaction: acyl-CoA + glycine < -- > CoA + N-acylglycine. The isolated excretion of 2-methylbutyrylglycine (2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase deficiency (PubMedID 15615815). A number of previously unrecognized abnormal metabolites have been identified and quantitated in the urine of patients with propionyl-CoA carboxylase deficiency. These include the isoleucine metabolites, 2-methyl-3-hydroxybutyric acid and 2-methylacetoacetic acid. Additionally, 3-hydroxyvaleric acid and 3-oxovaleric acid were found, which may be products of the condensation of propionyl-CoA with acetyl-CoA catalyzed by 3-oxoacyl-CoA thiolases. Following a load of isoleucine, 2-methylbutyrylglycine was identified. This metabolite has not previously been observed in man. (PMID: 630060) [HMDB] (2-methyl-butyrylamino)-acetate (2-methyl-butyrylamino)-acetic acid 2-MBG 2-Methylbutyryl glycine 2-Methylbutyrylglycine a-Methylbutyrylglycine alpha-Methylbutyrylglycine N-(2-Methylbutyryl)glycine N-sec-Valerylglycine C7H13NO3 159.183 159.089543287 2-(2-methylbutanamido)acetic acid 2-methylbutyrylglycine 52320-67-9 CCC(C)C(=O)NCC(O)=O InChI=1S/C7H13NO3/c1-3-5(2)7(11)8-4-6(9)10/h5H,3-4H2,1-2H3,(H,8,11)(H,9,10) HOACIBQKYRHBOW-UHFFFAOYSA-N belongs to the class of organic compounds known as n-acyl-alpha amino acids. N-acyl-alpha amino acids are compounds containing an alpha amino acid which bears an acyl group at its terminal nitrogen atom. N-acyl-alpha amino acids Organic compounds Organic acids and derivatives Carboxylic acids and derivatives Amino acids, peptides, and analogues Aliphatic acyclic compounds Carbonyl compounds Carboximidic acids Carboxylic acids Hydrocarbon derivatives Monocarboxylic acids and derivatives Organic oxides Organonitrogen compounds Organopnictogen compounds Propargyl-type 1,3-dipolar organic compounds Aliphatic acyclic compound Carbonyl group Carboximidic acid Carboximidic acid derivative Carboxylic acid Hydrocarbon derivative Monocarboxylic acid or derivatives N-acyl-alpha-amino acid Organic 1,3-dipolar compound Organic nitrogen compound Organic oxide Organic oxygen compound Organonitrogen compound Organooxygen compound Organopnictogen compound Propargyl-type 1,3-dipolar organic compound N-acylglycine Solid logp 0.41 ALOGPS logs -1.25 ALOGPS solubility 9.00e+00 g/l ALOGPS logp 0.36 ChemAxon pka_strongest_acidic 4.17 ChemAxon pka_strongest_basic -1.6 ChemAxon iupac 2-(2-methylbutanamido)acetic acid ChemAxon average_mass 159.183 ChemAxon mono_mass 159.089543287 ChemAxon smiles CCC(C)C(=O)NCC(O)=O ChemAxon formula C7H13NO3 ChemAxon inchi InChI=1S/C7H13NO3/c1-3-5(2)7(11)8-4-6(9)10/h5H,3-4H2,1-2H3,(H,8,11)(H,9,10) ChemAxon inchikey HOACIBQKYRHBOW-UHFFFAOYSA-N ChemAxon polar_surface_area 66.4 ChemAxon refractivity 39.25 ChemAxon polarizability 16.55 ChemAxon rotatable_bond_count 4 ChemAxon acceptor_count 3 ChemAxon donor_count 2 ChemAxon physiological_charge -1 ChemAxon formal_charge 0 ChemAxon Specdb::NmrTwoD 1279 Specdb::MsIr 427 Specdb::MsIr 428 Specdb::MsIr 429 Specdb::NmrOneD 1336 Specdb::NmrOneD 143550 Specdb::NmrOneD 143551 Specdb::NmrOneD 143552 Specdb::NmrOneD 143553 Specdb::NmrOneD 143554 Specdb::NmrOneD 143555 Specdb::NmrOneD 143556 Specdb::NmrOneD 143557 Specdb::NmrOneD 143558 Specdb::NmrOneD 143559 Specdb::NmrOneD 143560 Specdb::NmrOneD 143561 Specdb::NmrOneD 143562 Specdb::NmrOneD 143563 Specdb::NmrOneD 143564 Specdb::NmrOneD 143565 Specdb::NmrOneD 143566 Specdb::NmrOneD 143567 Specdb::NmrOneD 143568 Specdb::NmrOneD 143569 Specdb::CMs 19182 Specdb::CMs 37444 Specdb::CMs 136923 Specdb::CMs 144657 Specdb::CMs 1058918 Specdb::CMs 1058920 Specdb::CMs 1058921 Specdb::MsMs 546 Specdb::MsMs 547 Specdb::MsMs 548 Specdb::MsMs 308629 Specdb::MsMs 308630 Specdb::MsMs 308631 Specdb::MsMs 352858 Specdb::MsMs 352859 Specdb::MsMs 352860 Specdb::MsMs 2236251 Specdb::MsMs 2237145 Specdb::MsMs 2238387 Specdb::MsMs 2239174 Specdb::MsMs 2240410 Specdb::MsMs 2241314 Specdb::MsMs 2672366 Specdb::MsMs 2672367 Specdb::MsMs 2672368 Specdb::MsMs 3010338 Specdb::MsMs 3010339 Specdb::MsMs 3010340 HMDB00339 #<Reference:0x000055ce325560a8> #<Reference:0x000055ce32555ef0> #<Reference:0x000055ce32555d38> #<Reference:0x000055ce32555b80> 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 Glycine N-acyltransferase Q6IB77 GLYAT Glycine N-acyltransferase-like protein 1 Q969I3 GLYATL1 Glycine N-acyltransferase-like protein 2 Q8WU03 GLYATL2 Glycine N-acyltransferase-like protein 3 Q5SZD4 GLYATL3