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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-11-09 23:15:09 UTC
HMDB IDHMDB0000953
Secondary Accession Numbers
  • HMDB00953
Metabolite Identification
Common NameSuberylglycine
DescriptionSuberylglycine 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-acylglycineSuberylglycine is a dicarboxylic acid. It is formed by glycine-N-acylase catalyzed conjugation (PMID 947635 ). It can be used for the diagnosis of hereditary medium-chain acyl-CoA dehydrogenase deficiency (PMID 2775902 ). Suberylglycine is found to be associated with propionic acidemia, which is an inborn error of metabolism.
Structure
Data?1582752167
Synonyms
ValueSource
SuberylglycinHMDB
7-[(Carboxymethyl)-C-hydroxycarbonimidoyl]heptanoateHMDB
SuberylglycineMeSH
Chemical FormulaC10H17NO5
Average Molecular Weight231.2457
Monoisotopic Molecular Weight231.110672659
IUPAC Name7-[(carboxymethyl)carbamoyl]heptanoic acid
Traditional Name7-(carboxymethylcarbamoyl)heptanoic acid
CAS Registry Number60317-54-6
SMILES
OC(=O)CCCCCCC(=O)NCC(O)=O
InChI Identifier
InChI=1S/C10H17NO5/c12-8(11-7-10(15)16)5-3-1-2-4-6-9(13)14/h1-7H2,(H,11,12)(H,13,14)(H,15,16)
InChI KeyHXATVKDSYDWTCX-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
  • Medium-chain fatty acid
  • Amino fatty acid
  • Dicarboxylic acid or derivatives
  • Fatty amide
  • Fatty acyl
  • Fatty acid
  • N-acyl-amine
  • Carboxamide group
  • Secondary carboxylic acid amide
  • Carboxylic acid
  • Organic nitrogen compound
  • Organonitrogen compound
  • Organooxygen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
Physiological effect
Disposition
ProcessNot Available
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_neg146.81230932474
[M-H]-Not Available146.812http://allccs.zhulab.cn/database/detail?ID=AllCCS00000038
Predicted Molecular Properties
PropertyValueSource
Water Solubility2.02 g/LALOGPS
logP0.32ALOGPS
logP0.27ChemAxon
logS-2.1ALOGPS
pKa (Strongest Acidic)3.91ChemAxon
pKa (Strongest Basic)-1.7ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area103.7 ŲChemAxon
Rotatable Bond Count9ChemAxon
Refractivity54.74 m³·mol⁻¹ChemAxon
Polarizability23.96 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]+154.94231661259
DarkChem[M-H]-148.76831661259
AllCCS[M+H]+151.10332859911
AllCCS[M-H]-153.48832859911
DeepCCS[M+H]+151.45230932474
DeepCCS[M-H]-147.58930932474
DeepCCS[M-2H]-185.43830932474
DeepCCS[M+Na]+161.10230932474
AllCCS[M+H]+151.132859911
AllCCS[M+H-H2O]+147.732859911
AllCCS[M+NH4]+154.232859911
AllCCS[M+Na]+155.132859911
AllCCS[M-H]-153.532859911
AllCCS[M+Na-2H]-154.332859911
AllCCS[M+HCOO]-155.332859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
SuberylglycineOC(=O)CCCCCCC(=O)NCC(O)=O3238.5Standard polar33892256
SuberylglycineOC(=O)CCCCCCC(=O)NCC(O)=O1794.1Standard non polar33892256
SuberylglycineOC(=O)CCCCCCC(=O)NCC(O)=O2198.5Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Suberylglycine,1TMS,isomer #1C[Si](C)(C)OC(=O)CCCCCCC(=O)NCC(=O)O2113.5Semi standard non polar33892256
Suberylglycine,1TMS,isomer #2C[Si](C)(C)OC(=O)CNC(=O)CCCCCCC(=O)O2111.5Semi standard non polar33892256
Suberylglycine,1TMS,isomer #3C[Si](C)(C)N(CC(=O)O)C(=O)CCCCCCC(=O)O2139.2Semi standard non polar33892256
Suberylglycine,2TMS,isomer #1C[Si](C)(C)OC(=O)CCCCCCC(=O)NCC(=O)O[Si](C)(C)C2223.9Semi standard non polar33892256
Suberylglycine,2TMS,isomer #2C[Si](C)(C)OC(=O)CCCCCCC(=O)N(CC(=O)O)[Si](C)(C)C2183.1Semi standard non polar33892256
Suberylglycine,2TMS,isomer #3C[Si](C)(C)OC(=O)CN(C(=O)CCCCCCC(=O)O)[Si](C)(C)C2182.0Semi standard non polar33892256
Suberylglycine,3TMS,isomer #1C[Si](C)(C)OC(=O)CCCCCCC(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C2232.0Semi standard non polar33892256
Suberylglycine,3TMS,isomer #1C[Si](C)(C)OC(=O)CCCCCCC(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C2224.6Standard non polar33892256
Suberylglycine,3TMS,isomer #1C[Si](C)(C)OC(=O)CCCCCCC(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C2357.0Standard polar33892256
Suberylglycine,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CCCCCCC(=O)NCC(=O)O2393.0Semi standard non polar33892256
Suberylglycine,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)CNC(=O)CCCCCCC(=O)O2389.9Semi standard non polar33892256
Suberylglycine,1TBDMS,isomer #3CC(C)(C)[Si](C)(C)N(CC(=O)O)C(=O)CCCCCCC(=O)O2373.5Semi standard non polar33892256
Suberylglycine,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CCCCCCC(=O)NCC(=O)O[Si](C)(C)C(C)(C)C2709.0Semi standard non polar33892256
Suberylglycine,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)CCCCCCC(=O)N(CC(=O)O)[Si](C)(C)C(C)(C)C2678.1Semi standard non polar33892256
Suberylglycine,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)OC(=O)CN(C(=O)CCCCCCC(=O)O)[Si](C)(C)C(C)(C)C2675.5Semi standard non polar33892256
Suberylglycine,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CCCCCCC(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2938.6Semi standard non polar33892256
Suberylglycine,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CCCCCCC(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2777.3Standard non polar33892256
Suberylglycine,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CCCCCCC(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2716.1Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (Non-derivatized) - 70eV, Positivesplash10-0fki-5900000000-d8bdec7b825bfcdae6712017-09-01Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (2 TMS) - 70eV, Positivesplash10-0079-9372000000-042eee51859aad93b7c92017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (TMS_1_1) - 70eV, PositiveNot Available2021-11-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (TMS_1_3) - 70eV, PositiveNot Available2021-11-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (TMS_2_2) - 70eV, PositiveNot Available2021-11-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (TMS_2_3) - 70eV, PositiveNot Available2021-11-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (TBDMS_1_3) - 70eV, PositiveNot Available2021-11-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (TBDMS_2_1) - 70eV, PositiveNot Available2021-11-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (TBDMS_2_2) - 70eV, PositiveNot Available2021-11-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Suberylglycine GC-MS (TBDMS_2_3) - 70eV, PositiveNot Available2021-11-06Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-03di-0980000000-4eb03758ab16069cd6972012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-053r-9100000000-45599b39ba56ed1dbf682012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-0a4i-9000000000-60582ebad737b564619a2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine 20V, Negative-QTOFsplash10-00di-9200000000-b9751ab3dc30904853532021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine 40V, Negative-QTOFsplash10-00di-9000000000-b491fb926b44345e3ab12021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine 10V, Negative-QTOFsplash10-001i-3290000000-f864e3f27fa30fe2f93f2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine 10V, Positive-QTOFsplash10-0cdr-1920000000-4b7f4b02e1f8054d8a7e2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine 10V, Positive-QTOFsplash10-0cdr-2930000000-0768339e1f9a2f7823ed2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine 40V, Positive-QTOFsplash10-0a4i-9000000000-2f8123a3a79efd4131fe2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine 20V, Positive-QTOFsplash10-053r-9500000000-a98ac800d1bf1e9ef4502021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine 40V, Positive-QTOFsplash10-0a4i-9000000000-f5c9fa8ed6220d2b1fa72021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Suberylglycine 20V, Positive-QTOFsplash10-053r-9700000000-c37f5c0518cb9f46fad52021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 10V, Positive-QTOFsplash10-03di-3590000000-a5390db1a156d67988d72017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 20V, Positive-QTOFsplash10-056r-9610000000-91492ad2c4390d7ef68b2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 40V, Positive-QTOFsplash10-056r-9100000000-944ad13429ca8576fbcc2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 10V, Negative-QTOFsplash10-001i-0290000000-fc2161e02f12ea2a8c6a2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 20V, Negative-QTOFsplash10-06zi-5970000000-0e99e032d8c859237e5e2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 40V, Negative-QTOFsplash10-0ab9-9100000000-9944e8a5883f8b71a8f12017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 10V, Negative-QTOFsplash10-001i-1090000000-e804bd40e5e3352dc53b2021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 20V, Negative-QTOFsplash10-00di-9740000000-4a21c4038df64cbcf0612021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 40V, Negative-QTOFsplash10-00di-9000000000-284aeb654928fe9a314d2021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 10V, Positive-QTOFsplash10-003r-6390000000-c33d2bf0850eb0da350b2021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 20V, Positive-QTOFsplash10-05rr-9500000000-deb034db17fb88d153fb2021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Suberylglycine 40V, Positive-QTOFsplash10-053r-9000000000-fd26d3cdb072cd0ab5822021-09-24Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)2012-12-04Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum
Biological Properties
Cellular LocationsNot Available
Biospecimen Locations
  • Blood
  • 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
UrineDetected and Quantified0.0078–0.0681 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
UrineDetected and Quantified0.0035–0.0515 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
UrineDetected and Quantified<0.0020–0.0390 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
UrineDetected but not QuantifiedNot QuantifiedAdult (24-38years old)Not SpecifiedNormal details
UrineDetected and Quantified0.01-0.76 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified0-46.447 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • The analysis of d...
details
UrineDetected and Quantified1.467-30.802 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • The analysis of d...
details
UrineDetected and Quantified<0.2 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.0 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0.29 +/- 0.24 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified1.00 (0.00-2.00) umol/mmol creatinineAdult (>18 years old)BothMedium Chain Acyl-CoA Dehydrogenase Deficiency
    • MetaGene: Metabol...
details
UrineDetected and Quantified6.356-2226.0400 umol/mmol creatinineNewborn (0-30 days old)Both
Medium Chain Acyl-CoA Dehydrogenase Deficiency
    • The analysis of d...
details
UrineDetected and Quantified11.734-208.768 umol/mmol creatinineNewborn (0-30 days old)Both
Medium Chain Acyl-CoA Dehydrogenase Deficiency
    • The analysis of d...
details
UrineDetected and Quantified0.03 umol/mmol creatinineAdult (>18 years old)Not Specified
Propionic acidemia
details
UrineDetected and Quantified3.911 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified7.334 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified69.915 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified151.0754 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified352.509 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified98.0230-783.845 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedMedium Chain Acyl-CoA Dehydrogenase Deficiency details
Associated Disorders and Diseases
Disease References
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. Duran M, De Klerk JB, Wadman SK, Bruinvis L, Ketting D: The differential diagnosis of dicarboxylic aciduria. J Inherit Metab Dis. 1984;7 Suppl 1:48-51. [PubMed:6434845 ]
  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 ]
Associated OMIM IDs
  • 201450 (Medium Chain Acyl-CoA Dehydrogenase Deficiency)
  • 606054 (Propionic acidemia)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022336
KNApSAcK IDNot Available
Chemspider ID4956295
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID5899
PubChem Compound6453952
PDB IDNot Available
ChEBI IDNot Available
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00029991
Good Scents IDNot Available
References
Synthesis ReferenceGregersen, Niels; Gron, Ida; Rasmussen, Karsten; Kolvraa, Steen. Gas chromatographic mass spectrometric identification of N-dicarboxylmonoglycines. Biomedical Mass Spectrometry (1978), 5(1), 80-3.
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. Bhuiyan AK, Jackson S, Turnbull DM, Aynsley-Green A, Leonard JV, Bartlett K: The measurement of carnitine and acyl-carnitines: application to the investigation of patients with suspected inherited disorders of mitochondrial fatty acid oxidation. Clin Chim Acta. 1992 May 15;207(3):185-204. [PubMed:1327583 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
  6. Gregersen N, Winter V, Lyonnet S, Saudubray JM, Wendel U, Jensen TG, Andresen BS, Kolvraa S, Lehnert W, Bolund L, et al.: Molecular genetic characterization and urinary excretion pattern of metabolites in two families with MCAD deficiency due to compound heterozygosity with a 13 base pair insertion in one allele. J Inherit Metab Dis. 1994;17(2):169-84. [PubMed:7967471 ]
  7. Gregersen N, Lauritzen R, Rasmussen K: Suberylglycine excretion in the urine from a patient with dicarboxylic aciduria. Clin Chim Acta. 1976 Aug 2;70(3):417-25. [PubMed:947635 ]
  8. Tojo M, Gunji T, Yamaguchi S, Shimizu N, Koga Y, Nonaka I: [A case of riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II)]. No To Hattatsu. 2000 Mar;32(2):163-8. [PubMed:10723193 ]

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