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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-12-07 01:21:32 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
Thumb
SynonymsNot Available
Chemical FormulaC10H17NO5
Average Molecular Weight231.2457
Monoisotopic Molecular Weight231.110672659
IUPAC NameNot Available
Traditional NameNot Available
CAS Registry Number60317-54-6
SMILESNot Available
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
ClassificationNot classified
Ontology
Disposition

Biological Location:

  Biofluid and excreta:

Source:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted PropertiesNot Available
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0fki-5900000000-d8bdec7b825bfcdae671View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-0079-9372000000-042eee51859aad93b7c9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-03di-0980000000-4eb03758ab16069cd697View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-053r-9100000000-45599b39ba56ed1dbf68View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0a4i-9000000000-60582ebad737b564619aView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-03di-3590000000-a5390db1a156d67988d7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-056r-9610000000-91492ad2c4390d7ef68bView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-056r-9100000000-944ad13429ca8576fbccView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-001i-0290000000-fc2161e02f12ea2a8c6aView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-06zi-5970000000-0e99e032d8c859237e5eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0ab9-9100000000-9944e8a5883f8b71a8f1View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular LocationsNot Available
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
NameSMPDB/PathwhizKEGG
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not Quantified Not AvailableNot Available
Normal
    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 Quantified Adult (24-38years old)Not SpecifiedNormal 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 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
    BiofluidStatusValueAgeSexConditionReferenceDetails
    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. 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 ]
    2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
    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
    • 606054 (Propionic acidemia)
    • 201450 (Medium Chain Acyl-CoA Dehydrogenase Deficiency)
    DrugBank IDNot Available
    DrugBank Metabolite IDNot Available
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB 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
    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