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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-11-09 23:14:23 UTC
HMDB IDHMDB0000730
Secondary Accession Numbers
  • HMDB00730
Metabolite Identification
Common NameIsobutyrylglycine
DescriptionIsobutyrylglycine 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. Isobutyrylglycine is identified in large amount in urine of patients with isobutyryl-CoA dehydrogenase deficiency. Isobutyryl-CoA dehydrogenase deficiency is a disorder caused by the deficiency of isobutyryl-CoA dehydrogenase that is involved in the catabolism of the branched-chain amino acid valine (PMID 15505379 ). Moreover, Isobutyrylglycine is found to be associated with ethylmalonic encephalopathy and propionic acidemia, which are also inborn errors of metabolism. Isobutyrylglycine is a biomarker for the consumption of cheese.
Structure
Data?1582752152
Synonyms
ValueSource
IsobutanoylglycineChEBI
N-IsobutanoylglycineChEBI
N-(1-Methylpropyl)glycineHMDB
N-IsobutyrylglycineHMDB
IsobutyrylglycineChEBI
Chemical FormulaC6H11NO3
Average Molecular Weight145.1564
Monoisotopic Molecular Weight145.073893223
IUPAC Name2-(2-methylpropanamido)acetic acid
Traditional Nameisobutyrylglycine
CAS Registry Number15926-18-8
SMILES
CC(C)C(=O)NCC(O)=O
InChI Identifier
InChI=1S/C6H11NO3/c1-4(2)6(10)7-3-5(8)9/h4H,3H2,1-2H3,(H,7,10)(H,8,9)
InChI KeyDCICDMMXFIELDF-UHFFFAOYSA-N
Chemical Taxonomy
Description 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.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentN-acyl-alpha amino acids
Alternative Parents
Substituents
  • N-acyl-alpha-amino acid
  • Secondary carboxylic acid amide
  • Carboxamide group
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Adverse health effect

Disposition

Biological location

Source

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point82.5 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-MetCCS_train_neg132.06730932474
[M-H]-Not Available132.067http://allccs.zhulab.cn/database/detail?ID=AllCCS00000030
Predicted Molecular Properties
PropertyValueSource
Water Solubility17.9 g/LALOGPS
logP-0.11ALOGPS
logP-0.085ChemAxon
logS-0.91ALOGPS
pKa (Strongest Acidic)4.06ChemAxon
pKa (Strongest Basic)-1.8ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area66.4 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity34.65 m³·mol⁻¹ChemAxon
Polarizability14.57 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+132.07731661259
DarkChem[M-H]-127.54231661259
AllCCS[M+H]+133.42832859911
AllCCS[M-H]-130.87832859911
DeepCCS[M+H]+130.89830932474
DeepCCS[M-H]-127.36330932474
DeepCCS[M-2H]-164.29830932474
DeepCCS[M+Na]+139.46130932474

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
IsobutyrylglycineCC(C)C(=O)NCC(O)=O2295.0Standard polar33892256
IsobutyrylglycineCC(C)C(=O)NCC(O)=O1247.7Standard non polar33892256
IsobutyrylglycineCC(C)C(=O)NCC(O)=O1350.4Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Isobutyrylglycine,1TMS,isomer #1CC(C)C(=O)NCC(=O)O[Si](C)(C)C1380.6Semi standard non polar33892256
Isobutyrylglycine,1TMS,isomer #2CC(C)C(=O)N(CC(=O)O)[Si](C)(C)C1395.2Semi standard non polar33892256
Isobutyrylglycine,2TMS,isomer #1CC(C)C(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1437.7Semi standard non polar33892256
Isobutyrylglycine,2TMS,isomer #1CC(C)C(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1410.8Standard non polar33892256
Isobutyrylglycine,2TMS,isomer #1CC(C)C(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1547.3Standard polar33892256
Isobutyrylglycine,1TBDMS,isomer #1CC(C)C(=O)NCC(=O)O[Si](C)(C)C(C)(C)C1619.5Semi standard non polar33892256
Isobutyrylglycine,1TBDMS,isomer #2CC(C)C(=O)N(CC(=O)O)[Si](C)(C)C(C)(C)C1622.7Semi standard non polar33892256
Isobutyrylglycine,2TBDMS,isomer #1CC(C)C(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1874.6Semi standard non polar33892256
Isobutyrylglycine,2TBDMS,isomer #1CC(C)C(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1833.0Standard non polar33892256
Isobutyrylglycine,2TBDMS,isomer #1CC(C)C(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1832.0Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - Isobutyrylglycine GC-MS (Non-derivatized) - 70eV, Positivesplash10-00dl-9100000000-91e12bffc069cc1fe9a02017-09-01Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isobutyrylglycine GC-MS (1 TMS) - 70eV, Positivesplash10-00di-9100000000-5281458abfc09bd42ce12017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isobutyrylglycine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isobutyrylglycine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isobutyrylglycine GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isobutyrylglycine GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Isobutyrylglycine GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Isobutyrylglycine Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-00fr-9000000000-f1d95bb7599bb8f6cd732012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isobutyrylglycine Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-0006-9000000000-b41069b8dbfe4b85b3462012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isobutyrylglycine Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-0006-9000000000-85cb61c87f73b734a9e52012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isobutyrylglycine 35V, Positive-QTOFsplash10-0006-9000000000-3fc2c9bdad8d5402bc372021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Isobutyrylglycine 35V, Negative-QTOFsplash10-00di-9000000000-25d45687b4de12d7278a2021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 10V, Positive-QTOFsplash10-006t-4900000000-56e60896238c44e6c1632017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 20V, Positive-QTOFsplash10-00dl-9300000000-b49a0fcfcc97adc64a232017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 40V, Positive-QTOFsplash10-0596-9000000000-19f4499051d30aa160e82017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 10V, Negative-QTOFsplash10-0006-0900000000-96c02d5f830c0ad4ba522017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 20V, Negative-QTOFsplash10-0f6x-1900000000-be6409aa473bdd7dc4172017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 40V, Negative-QTOFsplash10-05fr-9000000000-2656458192decd1b1fbf2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 10V, Negative-QTOFsplash10-0006-0900000000-ea3f3c5f4359d8e7d6712021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 20V, Negative-QTOFsplash10-0kfw-9300000000-2add86a545e7ecd4a0ec2021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 40V, Negative-QTOFsplash10-0596-9000000000-d0a390fd6e37ef2d8a632021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 10V, Positive-QTOFsplash10-0006-9100000000-505e17d2533aeaf498f82021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 20V, Positive-QTOFsplash10-0006-9000000000-28a4e51c194d7751c7312021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Isobutyrylglycine 40V, Positive-QTOFsplash10-0006-9000000000-dda745bb6fc8ca1dced52021-09-24Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 500 MHz, CDCl3, experimental)2012-12-04Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, CD3OD, experimental)2012-12-05Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0-5 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected but not QuantifiedNot QuantifiedAdult (24-38years old)Not SpecifiedNormal details
UrineDetected and Quantified0.48 (0-1.92) umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified<2.9 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0.2157–1.6217 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
UrineDetected and Quantified0.1742–1.2490 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
UrineDetected and Quantified0.1643–0.8386 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
UrineDetected and Quantified<0.99 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<8.6 umol/mmol creatinineChildren (1 - 13 years old)FemaleNormal details
UrineDetected and Quantified0.5 - 3.8 umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified1.791-27.339 umol/mmol creatinineNewborn (0-30 days old)BothIsobutyryl-CoA Dehydrogenase Deficiency details
UrineDetected and Quantified1.558-7.477 umol/mmol creatinineInfant (0-1 year old)MaleIsobutyryl-CoA Dehydrogenase Deficiency details
UrineDetected and Quantified0.0234-1.106 umol/mmol creatinineNewborn (0-30 days old)Not SpecifiedShort Chain Acyl-Coa Dehydrogenase Deficiency details
UrineDetected and Quantified1.69 umol/mmol creatinineAdult (>18 years old)Not Specified
Propionic acidemia
details
UrineDetected and Quantified17.4 umol/mmol creatinineChildren (1 - 13 years old)FemaleEthylmalonic encephalopathy details
UrineDetected and Quantified31.155 umol/mmol creatinineNewborn (0-30 days old)Not SpecifiedGlutaric Aciduria II details
UrineDetected and Quantified4.5 - 33 umol/mmol creatinineAdolescent (13-18 years old)BothEthylmalonic encephalopathy details
Associated Disorders and Diseases
Disease References
Propionic acidemia
  1. Riemersma M, Hazebroek MR, Helderman-van den Enden ATJM, Salomons GS, Ferdinandusse S, Brouwers MCGJ, van der Ploeg L, Heymans S, Glatz JFC, van den Wijngaard A, Krapels IPC, Bierau J, Brunner HG: Propionic acidemia as a cause of adult-onset dilated cardiomyopathy. Eur J Hum Genet. 2017 Nov;25(11):1195-1201. doi: 10.1038/ejhg.2017.127. Epub 2017 Aug 30. [PubMed:28853722 ]
Ethylmalonic encephalopathy
  1. Nowaczyk MJ, Lehotay DC, Platt BA, Fisher L, Tan R, Phillips H, Clarke JT: Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism. Metabolism. 1998 Jul;47(7):836-9. [PubMed:9667231 ]
  2. Lehnert W, Ruitenbeek W: Ethylmalonic aciduria associated with progressive neurological disease and partial cytochrome c oxidase deficiency. J Inherit Metab Dis. 1993;16(3):557-9. [PubMed:7609451 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Glutaric aciduria II
  1. Gregersen N, Kolvraa S, Rasmussen K, Christensen E, Brandt NJ, Ebbesen F, Hansen FH: Biochemical studies in a patient with defects in the metabolism of acyl-CoA and sarcosine: another possible case of glutaric aciduria type II. J Inherit Metab Dis. 1980;3(3):67-72. [PubMed:6158623 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Isobutyryl-CoA Dehydrogenase Deficiency
  1. Oglesbee D, He M, Majumder N, Vockley J, Ahmad A, Angle B, Burton B, Charrow J, Ensenauer R, Ficicioglu CH, Keppen LD, Marsden D, Tortorelli S, Hahn SH, Matern D: Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency. Genet Med. 2007 Feb;9(2):108-16. doi: 10.1097GIM.0b013e31802f78d6. [PubMed:17304052 ]
Short Chain Acyl-Coa Dehydrogenase Deficiency
  1. Oglesbee D, He M, Majumder N, Vockley J, Ahmad A, Angle B, Burton B, Charrow J, Ensenauer R, Ficicioglu CH, Keppen LD, Marsden D, Tortorelli S, Hahn SH, Matern D: Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency. Genet Med. 2007 Feb;9(2):108-16. doi: 10.1097GIM.0b013e31802f78d6. [PubMed:17304052 ]
Associated OMIM IDs
  • 606054 (Propionic acidemia)
  • 602473 (Ethylmalonic encephalopathy)
  • 231680 (Glutaric aciduria II)
  • 611283 (Isobutyryl-CoA Dehydrogenase Deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022208
KNApSAcK IDC00052325
Chemspider ID9030891
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID5698
PubChem Compound10855600
PDB IDNot Available
ChEBI ID70979
Food Biomarker OntologyNot Available
VMH IDCE4969
MarkerDB IDMDB00000239
Good Scents IDNot Available
References
Synthesis ReferenceKatz, J.; Lieberman, I.; Barker, H. A. Formation of propionyl-, butyryl-, and other acylglycines by enzymes of Clostridium kluyveri. Journal of Biological Chemistry (1953), 200 431-41.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Sass JO, Sander S, Zschocke J: Isobutyryl-CoA dehydrogenase deficiency: isobutyrylglycinuria and ACAD8 gene mutations in two infants. J Inherit Metab Dis. 2004;27(6):741-5. [PubMed:15505379 ]
  2. Goodman SI, McCabe ER, Fennessey PV, Mace JW: Multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II) with transient hypersarcosinemia and sarcosinuria; possible inherited deficiency of an electron transfer flavoprotein. Pediatr Res. 1980 Jan;14(1):12-7. [PubMed:7360517 ]

Enzymes

General function:
Involved in glycine N-acyltransferase activity
Specific function:
Mitochondrial acyltransferase which transfers an acyl group to the N-terminus of glycine and glutamine, although much less efficiently. Can conjugate numerous substrates to form a variety of N-acylglycines, with a preference for benzoyl-CoA over phenylacetyl-CoA as acyl donors. Thereby detoxify xenobiotics, such as benzoic acid or salicylic acid, and endogenous organic acids, such as isovaleric acid.
Gene Name:
GLYAT
Uniprot ID:
Q6IB77
Molecular weight:
18506.33
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Acyltransferase which transfers an acyl group to the N-terminus of glutamine. Can use phenylacetyl-CoA as an acyl donor.
Gene Name:
GLYATL1
Uniprot ID:
Q969I3
Molecular weight:
35100.895
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Mitochondrial acyltransferase which transfers the acyl group to the N-terminus of glycine. Conjugates numerous substrates, such as arachidonoyl-CoA and saturated medium and long-chain acyl-CoAs ranging from chain-length C8:0-CoA to C18:0-CoA, to form a variety of N-acylglycines. Shows a preference for monounsaturated fatty acid oleoyl-CoA (C18:1-CoA) as an acyl donor. Does not exhibit any activity toward C22:6-CoA and chenodeoxycholoyl-CoA, nor toward serine or alanine.
Gene Name:
GLYATL2
Uniprot ID:
Q8WU03
Molecular weight:
34277.055
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Acyltransferase which transfers the acyl group to the N- terminus of glycine
Gene Name:
GLYATL3
Uniprot ID:
Q5SZD4
Molecular weight:
32703.3