1.02011-09-21 00:05:57 UTC2015-10-09 22:33:40 UTCFDB0219632-Methylbutyrylglycine2-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 acid2-MBG2-Methylbutyryl glycine2-Methylbutyrylglycinea-Methylbutyrylglycinealpha-MethylbutyrylglycineN-(2-Methylbutyryl)glycineN-sec-ValerylglycineC7H13NO3159.183159.0895432872-(2-methylbutanamido)acetic acid2-methylbutyrylglycine52320-67-9CCC(C)C(=O)NCC(O)=OInChI=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 acidsOrganic compoundsOrganic acids and derivativesCarboxylic acids and derivativesAmino acids, peptides, and analoguesAliphatic acyclic compoundsCarbonyl compoundsCarboximidic acidsCarboxylic acidsHydrocarbon derivativesMonocarboxylic acids and derivativesOrganic oxidesOrganonitrogen compoundsOrganopnictogen compoundsPropargyl-type 1,3-dipolar organic compoundsAliphatic acyclic compoundCarbonyl groupCarboximidic acidCarboximidic acid derivativeCarboxylic acidHydrocarbon derivativeMonocarboxylic acid or derivativesN-acyl-alpha-amino acidOrganic 1,3-dipolar compoundOrganic nitrogen compoundOrganic oxideOrganic oxygen compoundOrganonitrogen compoundOrganooxygen compoundOrganopnictogen compoundPropargyl-type 1,3-dipolar organic compoundN-acylglycineSolidlogp0.41logs-1.25solubility9.00e+00 g/llogp0.36pka_strongest_acidic4.17pka_strongest_basic-1.6iupac2-(2-methylbutanamido)acetic acidaverage_mass159.183mono_mass159.089543287smilesCCC(C)C(=O)NCC(O)=OformulaC7H13NO3inchiInChI=1S/C7H13NO3/c1-3-5(2)7(11)8-4-6(9)10/h5H,3-4H2,1-2H3,(H,8,11)(H,9,10)inchikeyHOACIBQKYRHBOW-UHFFFAOYSA-Npolar_surface_area66.4refractivity39.25polarizability16.55rotatable_bond_count4acceptor_count3donor_count2physiological_charge-1formal_charge0Specdb::NmrTwoD1279Specdb::MsIr427Specdb::MsIr428Specdb::MsIr429Specdb::CMs19182Specdb::CMs37444Specdb::CMs136923Specdb::CMs144657Specdb::CMs1058918Specdb::CMs1058920Specdb::CMs1058921Specdb::NmrOneD1336Specdb::NmrOneD143550Specdb::NmrOneD143551Specdb::NmrOneD143552Specdb::NmrOneD143553Specdb::NmrOneD143554Specdb::NmrOneD143555Specdb::NmrOneD143556Specdb::NmrOneD143557Specdb::NmrOneD143558Specdb::NmrOneD143559Specdb::NmrOneD143560Specdb::NmrOneD143561Specdb::NmrOneD143562Specdb::NmrOneD143563Specdb::NmrOneD143564Specdb::NmrOneD143565Specdb::NmrOneD143566Specdb::NmrOneD143567Specdb::NmrOneD143568Specdb::NmrOneD143569Specdb::MsMs546Specdb::MsMs547Specdb::MsMs548Specdb::MsMs308629Specdb::MsMs308630Specdb::MsMs308631Specdb::MsMs352858Specdb::MsMs352859Specdb::MsMs352860Specdb::MsMs2236251Specdb::MsMs2237145Specdb::MsMs2238387Specdb::MsMs2239174Specdb::MsMs2240410Specdb::MsMs2241314Specdb::MsMs2672366Specdb::MsMs2672367Specdb::MsMs2672368Specdb::MsMs3010338Specdb::MsMs3010339Specdb::MsMs3010340HMDB00339#<Reference:0x00005556745d1ae0>#<Reference:0x00005556745d18b0>#<Reference:0x00005556745d16f8>#<Reference:0x00005556745d1540>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