1.02011-09-21 00:09:36 UTC2017-10-04 21:30:24 UTCFDB022177IsovalerylglycineIsovalerylglycine 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 acid2-(3-methylbutanoylamino)ethanoate2-(3-methylbutanoylamino)ethanoic acid3-methylbutyrylglycineIsopentanoylglycineIsovalerylglycineN-IsopentanoylglycineN-IsovaleroylglycineN-isovaleryl-glycineC7H13NO3159.183159.0895432872-(3-methylbutanamido)acetic acidisovalerylglycine16284-60-9CC(C)CC(=O)NCC(O)=OInChI=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 acidsOrganic compoundsOrganic acids and derivativesCarboxylic acids and derivativesAmino acids, peptides, and analoguesAliphatic acyclic compoundsCarbonyl compoundsCarboxylic acidsHydrocarbon derivativesMonocarboxylic acids and derivativesN-acyl aminesOrganic oxidesOrganonitrogen compoundsOrganopnictogen compoundsSecondary carboxylic acid amidesAliphatic acyclic compoundCarbonyl groupCarboxamide groupCarboxylic acidFatty acylFatty amideHydrocarbon derivativeMonocarboxylic acid or derivativesN-acyl-alpha-amino acidN-acyl-amineOrganic nitrogen compoundOrganic oxideOrganic oxygen compoundOrganonitrogen compoundOrganooxygen compoundOrganopnictogen compoundSecondary carboxylic acid amideN-acylglycineSolidlogp0.35logs-1.39solubility6.50e+00 g/llogp0.1pka_strongest_acidic4.17pka_strongest_basic-1.7iupac2-(3-methylbutanamido)acetic acidaverage_mass159.183mono_mass159.089543287smilesCC(C)CC(=O)NCC(O)=OformulaC7H13NO3inchiInChI=1S/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11)inchikeyZRQXMKMBBMNNQC-UHFFFAOYSA-Npolar_surface_area66.4refractivity39.22polarizability16.4rotatable_bond_count4acceptor_count3donor_count2physiological_charge-1formal_charge0Specdb::MsIr730Specdb::MsIr731Specdb::MsIr732Specdb::NmrTwoD1419Specdb::CMs25803Specdb::CMs37692Specdb::CMs159108Specdb::CMs1072399Specdb::CMs1072401Specdb::CMs1072403Specdb::NmrOneD1474Specdb::NmrOneD7362Specdb::NmrOneD7363Specdb::NmrOneD7364Specdb::NmrOneD7365Specdb::NmrOneD7366Specdb::NmrOneD7367Specdb::NmrOneD7368Specdb::NmrOneD7369Specdb::NmrOneD7370Specdb::NmrOneD7371Specdb::NmrOneD7372Specdb::NmrOneD7373Specdb::NmrOneD7374Specdb::NmrOneD7375Specdb::NmrOneD7376Specdb::NmrOneD7377Specdb::NmrOneD7378Specdb::NmrOneD7379Specdb::NmrOneD7380Specdb::NmrOneD7381Specdb::MsMs939Specdb::MsMs940Specdb::MsMs941Specdb::MsMs323200Specdb::MsMs323201Specdb::MsMs323202Specdb::MsMs371299Specdb::MsMs371300Specdb::MsMs371301Specdb::MsMs2238597Specdb::MsMs2239923Specdb::MsMs2241773Specdb::MsMs2243903Specdb::MsMs2244898Specdb::MsMs2245202Specdb::MsMs2246973Specdb::MsMs2247335Specdb::MsMs2248938Specdb::MsMs2249437Specdb::MsMs2251417Specdb::MsMs2465559Specdb::MsMs2465560Specdb::MsMs2465561Specdb::MsMs2496881Specdb::MsMs2496882HMDB00678#<Reference:0x000055567620b3a0>#<Reference:0x000055567620afb8>#<Reference:0x000055567620ad60>#<Reference:0x000055567620ab58>AnatidaeType 1specificAnatidae8830BeefaloType 1specificBos taurus X Bison bison297284BisonType 1specificBison bison9901BuffaloType 1specificBubalus bubalis89462Cattle (Beef, Veal)Type 1specificBos taurus9913ChickenType 1specificGallus gallus9031Columbidae (Dove, Pigeon)Type 1specificColumbidae8930DeerType 1specificCervidae9850Domestic goatType 1specificCapra aegagrus hircus9925Domestic pigType 1specificSus scrofa domestica9825ElkType 1specificCervus canadensis1574408EmuType 1specificDromaius novaehollandiae8790European rabbitType 1specificOryctolagus9984Greylag gooseType 1specificAnser anser8843Guinea henType 1specificNumida meleagris8996HorseType 1specificEquus caballus9796Mallard duckType 1specificAnas platyrhynchos8839Mountain hareType 1specificLepus timidus62621Mule deerType 1specificOdocoileus9871OstrichType 1specificStruthio camelus8801PheasantType 1specificPhasianus colchicus9054QuailType 1specificPhasianidae9005RabbitType 1specificLeporidae9979Rock ptarmiganType 1specificLagopus muta64668Sheep (Mutton, Lamb)Type 1specificOvis aries9940SquabType 1specificColumba8931TurkeyType 1specificMeleagris gallopavo9103Velvet duckType 1specificMelanitta fusca371864Wild boarType 1specificSus scrofa9823Glycine N-acyltransferaseQ6IB77GLYATGlycine N-acyltransferase-like protein 1Q969I3GLYATL1Glycine N-acyltransferase-like protein 2Q8WU03GLYATL2Glycine N-acyltransferase-like protein 3Q5SZD4GLYATL3