1.0 2011-09-21 00:09:36 UTC 2024-11-29 22:27:17 UTC FDB022177 Isovalerylglycine Isovalerylglycine 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 Isovalerylglycine is a byproduct of the catabolism of the aminoacid leucine. Accumulation of isovalerylglycine occurs in Isovaleric Acidemia (IVA). IVA (OMIM/ McKusick 243500) is an autosomal recessive disorder caused by mutations in the isovaleryl-CoA dehydrogenase (EC 1.3.99.10) gene. The deficiency of this enzyme in the metabolism of leucine leads to the accumulation of a series of isovaleryl-CoA metabolites, such as isovalerylglycine (IVG). It is very important to caution for false positive results when screening for isovaleric acidemia by tandem mass spectrometry based on dried blood-spot levels of C5-acylcarnitines, including isovalerylcarnitine and its isomer, pivaloylcarnitine; pivaloylcarnitine is derived from pivalate-generating antibiotics, and has caused many false-positive results. (PMID: 17850781) [HMDB]. Isovalerylglycine is a biomarker for the consumption of cheese. [(3-methylbutanoyl)amino]acetate [(3-methylbutanoyl)amino]acetic acid 2-(3-methylbutanoylamino)ethanoate 2-(3-methylbutanoylamino)ethanoic acid 3-methylbutyrylglycine Isopentanoylglycine Isovalerylglycine N-Isopentanoylglycine N-Isovaleroylglycine N-isovaleryl-glycine C7H13NO3 159.183 159.089543287 2-(3-methylbutanamido)acetic acid isovalerylglycine 16284-60-9 CC(C)CC(=O)NCC(O)=O InChI=1S/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11) ZRQXMKMBBMNNQC-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 Carboxylic acids Hydrocarbon derivatives Monocarboxylic acids and derivatives N-acyl amines Organic oxides Organonitrogen compounds Organopnictogen compounds Secondary carboxylic acid amides Aliphatic acyclic compound Carbonyl group Carboxamide group Carboxylic acid Fatty acyl Fatty amide Hydrocarbon derivative Monocarboxylic acid or derivatives N-acyl-alpha-amino acid N-acyl-amine Organic nitrogen compound Organic oxide Organic oxygen compound Organonitrogen compound Organooxygen compound Organopnictogen compound Secondary carboxylic acid amide N-acylglycine Solid logp 0.35 ALOGPS logs -1.39 ALOGPS solubility 6.50e+00 g/l ALOGPS logp 0.1 ChemAxon pka_strongest_acidic 4.17 ChemAxon pka_strongest_basic -1.7 ChemAxon iupac 2-(3-methylbutanamido)acetic acid ChemAxon average_mass 159.183 ChemAxon mono_mass 159.089543287 ChemAxon smiles CC(C)CC(=O)NCC(O)=O ChemAxon formula C7H13NO3 ChemAxon inchi InChI=1S/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11) ChemAxon inchikey ZRQXMKMBBMNNQC-UHFFFAOYSA-N ChemAxon polar_surface_area 66.4 ChemAxon refractivity 39.22 ChemAxon polarizability 16.4 ChemAxon rotatable_bond_count 4 ChemAxon acceptor_count 3 ChemAxon donor_count 2 ChemAxon physiological_charge -1 ChemAxon formal_charge 0 ChemAxon Metabolism and Physiological Effects of Isovalerylglycine SMP0126748 Specdb::MsIr 730 Specdb::MsIr 731 Specdb::MsIr 732 Specdb::NmrTwoD 1419 Specdb::CMs 25803 Specdb::CMs 37692 Specdb::CMs 159108 Specdb::CMs 1072399 Specdb::CMs 1072401 Specdb::CMs 1072403 Specdb::MsMs 939 Specdb::MsMs 940 Specdb::MsMs 941 Specdb::MsMs 323200 Specdb::MsMs 323201 Specdb::MsMs 323202 Specdb::MsMs 371299 Specdb::MsMs 371300 Specdb::MsMs 371301 Specdb::MsMs 2238597 Specdb::MsMs 2239923 Specdb::MsMs 2241773 Specdb::MsMs 2243903 Specdb::MsMs 2244898 Specdb::MsMs 2245202 Specdb::MsMs 2246973 Specdb::MsMs 2247335 Specdb::MsMs 2248938 Specdb::MsMs 2249437 Specdb::MsMs 2251417 Specdb::MsMs 2465559 Specdb::MsMs 2465560 Specdb::MsMs 2465561 Specdb::MsMs 2496881 Specdb::MsMs 2496882 Specdb::NmrOneD 1474 Specdb::NmrOneD 7362 Specdb::NmrOneD 7363 Specdb::NmrOneD 7364 Specdb::NmrOneD 7365 Specdb::NmrOneD 7366 Specdb::NmrOneD 7367 Specdb::NmrOneD 7368 Specdb::NmrOneD 7369 Specdb::NmrOneD 7370 Specdb::NmrOneD 7371 Specdb::NmrOneD 7372 Specdb::NmrOneD 7373 Specdb::NmrOneD 7374 Specdb::NmrOneD 7375 Specdb::NmrOneD 7376 Specdb::NmrOneD 7377 Specdb::NmrOneD 7378 Specdb::NmrOneD 7379 Specdb::NmrOneD 7380 Specdb::NmrOneD 7381 HMDB00678 #<Reference:0x000055ce315bbbc0> #<Reference:0x000055ce315bb8f0> #<Reference:0x000055ce315bb648> #<Reference:0x000055ce315bb350> 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