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Record Information
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:03:36 UTC
Secondary Accession NumbersNone
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 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 ).
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
InChI Identifier
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as n-acyl-aliphatic-alpha amino acids. These are alpha amino acids carrying a N-acylated aliphatic chain.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentN-acyl-aliphatic-alpha amino acids
Alternative Parents
  • N-acyl-aliphatic-alpha amino acid
  • Medium-chain fatty acid
  • Amino fatty acid
  • Fatty acyl
  • Fatty acid
  • N-acyl-amine
  • Fatty amide
  • Dicarboxylic acid or derivatives
  • Secondary carboxylic acid amide
  • Carboxamide group
  • Carboxylic acid
  • Carboxylic acid amide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
StatusDetected and Quantified
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locationsNot Available
Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility2.02 mg/mLALOGPS
pKa (Strongest Acidic)3.91ChemAxon
pKa (Strongest Basic)-0.95ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area103.7 Å2ChemAxon
Rotatable Bond Count9ChemAxon
Refractivity54.74 m3·mol-1ChemAxon
Polarizability23.96 Å3ChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectrum TypeDescriptionSplash Key
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
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular LocationsNot Available
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BloodExpected but not QuantifiedNot ApplicableNot AvailableNot Available
  • Not Applicable
UrineDetected and Quantified0.0 umol/mmol creatinineChildren (1 - 18 years old)Both
    • BC Children's Hos...
UrineDetected and Quantified0.29 +/- 0.24 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
Abnormal Concentrations
UrineDetected and Quantified1.00 (0.00-2.00) umol/mmol creatinineAdult (>18 years old)BothMedium Chain Acyl-CoA Dehydrogenase Deficiency details
UrineDetected and Quantified1100.00 (6.00-2200.00) umol/mmol creatinineChildren (1-13 years old)BothMedium chain acyl-CoA dehydrogenase deficiency (MCAD) details
Associated Disorders and Diseases
Disease References
Medium Chain Acyl-CoA Dehydrogenase Deficiency
  1. MetaGene [Link]
Associated OMIM IDs
  • 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
NuGOwiki LinkHMDB00953
Metagene LinkHMDB00953
PubChem Compound6453952
PDB IDNot Available
ChEBI IDNot Available
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. [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. [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. [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. [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. [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. [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. [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. [10723193 ]


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:
Uniprot ID:
Molecular weight:
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:
Uniprot ID:
Molecular weight:
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:
Uniprot ID:
Molecular weight:
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Acyltransferase which transfers the acyl group to the N- terminus of glycine
Gene Name:
Uniprot ID:
Molecular weight: