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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:15:04 UTC
HMDB IDHMDB0000701
Secondary Accession Numbers
  • HMDB00701
Metabolite Identification
Common NameHexanoylglycine
DescriptionHexanoylglycine 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-acylglycineHexanoylglycine is a fatty acid metabolite, it appears in the urine of patients with hereditary medium-chain acyl-CoA dehydrogenase (MCAD) deficiency (PMID 2775902 ), which is a genetic disorder.
Structure
Data?1676999704
Synonyms
ValueSource
CaproylglycineChEBI
N-(1-Oxohexyl)glycineChEBI
N-CaproylglycineChEBI
N-Hexanoyl-glycineHMDB
N-HexanoylglycineHMDB
HexanoylglycineChEBI
Chemical FormulaC8H15NO3
Average Molecular Weight173.2096
Monoisotopic Molecular Weight173.105193351
IUPAC Name2-hexanamidoacetic acid
Traditional Namehexanoylglycine
CAS Registry Number24003-67-6
SMILES
CCCCCC(=O)NCC(O)=O
InChI Identifier
InChI=1S/C8H15NO3/c1-2-3-4-5-7(10)9-6-8(11)12/h2-6H2,1H3,(H,9,10)(H,11,12)
InChI KeyUPCKIPHSXMXJOX-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
  • Carboximidic acid
  • Carboximidic acid derivative
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic 1,3-dipolar compound
  • Propargyl-type 1,3-dipolar organic compound
  • Carbonyl group
  • Organooxygen compound
  • Organonitrogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Organic nitrogen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Disposition
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot 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_neg144.63830932474
[M-H]-Not Available144.638http://allccs.zhulab.cn/database/detail?ID=AllCCS00000026
Predicted Molecular Properties
PropertyValueSource
Water Solubility3.61 g/LALOGPS
logP0.9ALOGPS
logP0.71ChemAxon
logS-1.7ALOGPS
pKa (Strongest Acidic)4.25ChemAxon
pKa (Strongest Basic)-1.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area66.4 ŲChemAxon
Rotatable Bond Count6ChemAxon
Refractivity43.88 m³·mol⁻¹ChemAxon
Polarizability18.75 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+140.3731661259
DarkChem[M-H]-137.62731661259
AllCCS[M+H]+140.9432859911
AllCCS[M-H]-140.43832859911
DeepCCS[M+H]+138.71830932474
DeepCCS[M-H]-134.95530932474
DeepCCS[M-2H]-172.30330932474
DeepCCS[M+Na]+147.5530932474
AllCCS[M+H]+140.932859911
AllCCS[M+H-H2O]+137.132859911
AllCCS[M+NH4]+144.532859911
AllCCS[M+Na]+145.532859911
AllCCS[M-H]-140.432859911
AllCCS[M+Na-2H]-142.132859911
AllCCS[M+HCOO]-144.032859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
HexanoylglycineCCCCCC(=O)NCC(O)=O2512.4Standard polar33892256
HexanoylglycineCCCCCC(=O)NCC(O)=O1457.4Standard non polar33892256
HexanoylglycineCCCCCC(=O)NCC(O)=O1626.7Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Hexanoylglycine,1TMS,isomer #1CCCCCC(=O)NCC(=O)O[Si](C)(C)C1612.9Semi standard non polar33892256
Hexanoylglycine,1TMS,isomer #2CCCCCC(=O)N(CC(=O)O)[Si](C)(C)C1607.0Semi standard non polar33892256
Hexanoylglycine,2TMS,isomer #1CCCCCC(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1652.1Semi standard non polar33892256
Hexanoylglycine,2TMS,isomer #1CCCCCC(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1673.1Standard non polar33892256
Hexanoylglycine,2TMS,isomer #1CCCCCC(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1808.0Standard polar33892256
Hexanoylglycine,1TBDMS,isomer #1CCCCCC(=O)NCC(=O)O[Si](C)(C)C(C)(C)C1840.8Semi standard non polar33892256
Hexanoylglycine,1TBDMS,isomer #2CCCCCC(=O)N(CC(=O)O)[Si](C)(C)C(C)(C)C1833.3Semi standard non polar33892256
Hexanoylglycine,2TBDMS,isomer #1CCCCCC(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2107.7Semi standard non polar33892256
Hexanoylglycine,2TBDMS,isomer #1CCCCCC(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2066.4Standard non polar33892256
Hexanoylglycine,2TBDMS,isomer #1CCCCCC(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2076.6Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - Hexanoylglycine GC-MS (Non-derivatized) - 70eV, Positivesplash10-0fdo-9300000000-d63753a58cbed9d64a0b2017-09-01Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Hexanoylglycine GC-MS (1 TMS) - 70eV, Positivesplash10-00di-9100000000-73a7c4329504ed7057112017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Hexanoylglycine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Hexanoylglycine GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Hexanoylglycine GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Hexanoylglycine 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 - Hexanoylglycine 20V, Negative-QTOFsplash10-00di-9000000000-c2c7c6516a4c26005a3e2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Hexanoylglycine 40V, Negative-QTOFsplash10-00di-9000000000-8edbd20ffcad8e974ad82021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Hexanoylglycine 10V, Negative-QTOFsplash10-00di-9200000000-0f1cc56efc88b769b2022021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Hexanoylglycine Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-004i-9000000000-ae0fac4a19a715861e4a2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Hexanoylglycine Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-0006-9000000000-0cd5551af50bb9dab1022012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Hexanoylglycine Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-0006-9000000000-7b2d1ccb3cfd6637ba942012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Hexanoylglycine 40V, Positive-QTOFsplash10-0006-9000000000-8ee973dbde07ab2dc7e32021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Hexanoylglycine 20V, Positive-QTOFsplash10-0006-9000000000-0ac5f3602586aca9837a2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Hexanoylglycine 10V, Positive-QTOFsplash10-00ba-9000000000-146c8038a60ab4b8bbb82021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 10V, Negative-QTOFsplash10-00di-0900000000-1b550dc2062741e683812017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 20V, Negative-QTOFsplash10-00di-5900000000-4581a83fc51b5f9822452017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 40V, Negative-QTOFsplash10-05fu-9000000000-0bc8374f98e4c05669402017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 10V, Negative-QTOFsplash10-00di-9700000000-62b9b5a0f8d79da36e222021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 20V, Negative-QTOFsplash10-00di-9100000000-c7d2139b876ee8d24df42021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 40V, Negative-QTOFsplash10-00di-9000000000-0aea0bfd802824f29c532021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 10V, Positive-QTOFsplash10-00fr-6900000000-4965ec5d947be1bb6c1d2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 20V, Positive-QTOFsplash10-056r-9200000000-1efeb9e53e85b74b6b652017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 40V, Positive-QTOFsplash10-0adl-9000000000-71afd16555594867855f2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 10V, Positive-QTOFsplash10-004i-9200000000-318791dd01dc2494e0702021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 20V, Positive-QTOFsplash10-00fr-9000000000-33d20565f405cf67d01f2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Hexanoylglycine 40V, Positive-QTOFsplash10-0006-9000000000-30720ba72ba1f9743ab22021-09-22Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 500 MHz, CDCl3, experimental)2012-12-04Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 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, 1000 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, 200 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, 300 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, 400 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, 500 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, 600 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, 700 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, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, CDCl3, experimental)2012-12-05Wishart LabView Spectrum

IR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)2023-02-03FELIX labView Spectrum
Biological Properties
Cellular Locations
  • Membrane
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot QuantifiedNot AvailableNot Available
Normal
      Not Available
details
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
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.0213–0.1990 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
UrineDetected and Quantified0.0268–0.3753 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
UrineDetected and Quantified0.0338–0.1983 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
UrineDetected but not QuantifiedNot QuantifiedAdult (24-38years old)Not SpecifiedNormal details
UrineDetected and Quantified0.03-0.62 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified1.30-13.06 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • The analysis of d...
details
UrineDetected and Quantified14.360-70.495 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • The analysis of d...
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified<2.88 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified<0.39 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<1 umol/mmol creatinineChildren (1 - 13 years old)BothNormal details
UrineDetected and Quantified<11.7 umol/mmol creatinineChildren (1 - 13 years old)FemaleNormal details
UrineDetected and Quantified0.900 +/- 0.400 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified0.41 +/- 1.72 umol/mmol creatinineInfant (0-1 year old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
UrineDetected and Quantified18.277-97.910 umol/mmol creatinineNewborn (0-30 days old)Both
Medium Chain Acyl-CoA Dehydrogenase Deficiency
    • The analysis of d...
details
UrineDetected and Quantified1.958-426.236 umol/mmol creatinineNewborn (0-30 days old)Both
Medium Chain Acyl-CoA Dehydrogenase Deficiency
    • The analysis of d...
details
UrineDetected and Quantified0.08 umol/mmol creatinineAdult (>18 years old)Not Specified
Propionic acidemia
details
UrineDetected and Quantified2.8 umol/mmol creatinineChildren (1 - 13 years old)FemaleEthylmalonic encephalopathy details
UrineDetected and Quantified5.222 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified26.762 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified57.441 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified59.399 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified114.881 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified0-3.0679 umol/mmol creatinineInfant (0-1 year old)Both
Short chain acyl-CoA dehydrogenase deficiency (SCAD)
details
UrineDetected and Quantified10-169 umol/mmol creatinineChildren (1 - 13 years old)BothMedium Chain Acyl-CoA Dehydrogenase Deficiency details
Associated Disorders and Diseases
Disease References
Colorectal cancer
  1. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Medium Chain Acyl-CoA Dehydrogenase Deficiency
  1. Tserng KY, Jin SJ, Kerr DS, Hoppel CL: Abnormal urinary excretion of unsaturated dicarboxylic acids in patients with medium-chain acyl-CoA dehydrogenase deficiency. J Lipid Res. 1990 May;31(5):763-71. [PubMed:2380628 ]
  2. Gregersen N, Kolvraa S, Rasmussen K, Mortensen PB, Divry P, David M, Hobolth N: General (medium-chain) acyl-CoA dehydrogenase deficiency (non-ketotic dicarboxylic aciduria): quantitative urinary excretion pattern of 23 biologically significant organic acids in three cases. Clin Chim Acta. 1983 Aug 15;132(2):181-91. [PubMed:6616873 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
  4. Millington D, Kodo N, Terada N, Roe C, Chace D (1991). The analysis of diagnostic markers of genetic disorders in human blood and urine using tandem mass spectrometry with liquid secondary ion mass spectrometry. Int J Mass Spectrom Ion Proc. 1991;111:211-228. Int J Mass Spectrom Ion Proc..
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 ]
Short Chain Acyl-Coa Dehydrogenase Deficiency
  1. Bhala A, Willi SM, Rinaldo P, Bennett MJ, Schmidt-Sommerfeld E, Hale DE: Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr. 1995 Jun;126(6):910-5. [PubMed:7776094 ]
Associated OMIM IDs
  • 114500 (Colorectal cancer)
  • 201450 (Medium Chain Acyl-CoA Dehydrogenase Deficiency)
  • 606054 (Propionic acidemia)
  • 602473 (Ethylmalonic encephalopathy)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022190
KNApSAcK IDNot Available
Chemspider ID89859
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID5669
PubChem Compound99463
PDB IDNot Available
ChEBI ID64390
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00000227
Good Scents IDNot Available
References
Synthesis ReferenceToth, Eugene; Weiss, Benjamine; Banay-Schwartz, Miriam; Lajtha, Abel. Effect of glycine derivatives on behavioral changes induced by 3-mercaptopropionic acid or phencyclidine in mice. Research Communications in Psychology, Psychiatry and Behavior (1986), 11(1), 1-9.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed:2026685 ]
  2. Rinaldo P, O'Shea JJ, Welch RD, Tanaka K: Stable isotope dilution analysis of n-hexanoylglycine, 3-phenylpropionylglycine and suberylglycine in human urine using chemical ionization gas chromatography/mass spectrometry selected ion monitoring. Biomed Environ Mass Spectrom. 1989 Jul;18(7):471-7. [PubMed:2775902 ]
  3. Chabrol B, Mancini J, Bertrand C, Vianey-Saban C, Divry P, Livet MO, Pinsard N: [Generalized epilepsy disclosing medium-chain-acyl-CoA dehydrogenase deficiency]. Arch Fr Pediatr. 1993 Jun-Jul;50(6):497-500. [PubMed:8135611 ]
  4. Tserng KY, Jin SJ, Kerr DS, Hoppel CL: Abnormal urinary excretion of unsaturated dicarboxylic acids in patients with medium-chain acyl-CoA dehydrogenase deficiency. J Lipid Res. 1990 May;31(5):763-71. [PubMed:2380628 ]
  5. Yamaguchi S, Shimizu N, Orii T, Fukao T, Suzuki Y, Maeda K, Hashimoto T, Previs SF, Rinaldo P: Prenatal diagnosis and neonatal monitoring of a fetus with glutaric aciduria type II due to electron transfer flavoprotein (beta-subunit) deficiency. Pediatr Res. 1991 Nov;30(5):439-43. [PubMed:1754299 ]

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