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Record Information
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-08-16 03:30:25 UTC
Secondary Accession Numbers
  • HMDB00860
Metabolite Identification
Common NamePhenylpropionylglycine
DescriptionPhenylpropionylglycine 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-acylglycineThe detection of phenylpropionylglycine in urine after an oral load of phenylpropionic acid can be used to diagnose deficiency of medium-chain acyl-CoA dehydrogenase, a frequent and treatable metabolic defect. (PMID 9234867 ).
(3-Phenyl-propionylamino)-acetic acidHMDB
Chemical FormulaC11H13NO3
Average Molecular Weight207.2258
Monoisotopic Molecular Weight207.089543287
IUPAC Name2-(3-phenylpropanamido)acetic acid
Traditional Namephenylpropionylglycine
CAS Registry Number56613-60-6
InChI Identifier
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as n-acyl-alpha amino acids. These are compounds containing an alpha amino acid which bears an acyl group at its terminal nitrogen atom.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganic acids and derivatives
Sub ClassCarboxylic acids and derivatives
Direct ParentN-acyl-alpha amino acids
Alternative Parents
  • N-acyl-alpha-amino acid
  • Monocyclic benzene moiety
  • Fatty amide
  • Fatty acyl
  • Benzenoid
  • Carboxamide group
  • Secondary carboxylic acid amide
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic nitrogen compound
  • Organonitrogen compound
  • Organooxygen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Carbonyl group
  • Aromatic homomonocyclic compound
Molecular FrameworkAromatic homomonocyclic 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 Solubility0.25 mg/mLALOGPS
pKa (Strongest Acidic)4ChemAxon
pKa (Strongest Basic)-1.5ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area66.4 Å2ChemAxon
Rotatable Bond Count5ChemAxon
Refractivity54.77 m3·mol-1ChemAxon
Polarizability21.48 Å3ChemAxon
Number of Rings1ChemAxon
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)splash10-056r-9510000000-857882337db8707be6daView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0a4i-3900000000-ac64df3f4f25d003198bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-056u-9400000000-ba5317e2dcb281f5ad7fView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular LocationsNot Available
Biofluid Locations
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
UrineDetected and Quantified<0.0010–0.0656 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
UrineDetected and Quantified<0.0010–0.0657 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
UrineDetected and Quantified0.0020–0.0573 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
UrineDetected but not Quantified Adult (24-38years old)Not SpecifiedNormal details
UrineDetected and Quantified0.0 umol/mmol creatinineChildren (1 - 18 years old)Both
    • BC Children's Hos...
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 Quantified45.5 (1.00-90.00) 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 IDFDB022286
KNApSAcK IDNot Available
Chemspider ID134261
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB0000860
PubChem Compound152323
PDB IDNot Available
ChEBI ID266653
Synthesis ReferenceGeurts, Muriel; Poupaert, Jacques H.; Scriba, Gerhard K. E.; Lambert, Didier M. N-(Benzyloxycarbonyl)glycine Esters and Amides as New Anticonvulsants. Journal of Medicinal Chemistry (1998), 41(1), 24-30.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Green A, Preece MA, de Sousa C, Pollitt RJ: Possible deleterious effect of L-carnitine supplementation in a patient with mild multiple acyl-CoA dehydrogenation deficiency (ethylmalonic-adipic aciduria). J Inherit Metab Dis. 1991;14(5):691-7. [PubMed:1779616 ]
  2. Rocchiccioli F, Cartier PH, Bougneres PF: Mass spectrometric identification of abnormal aromatic compounds in the urine of a child with Reye's like syndrome. Biomed Mass Spectrom. 1984 Mar;11(3):127-31. [PubMed:6722284 ]
  3. Flath B, Rolinski B, Roscher AA: Simple high-performance liquid chromatographic method for the detection of phenylpropionylglycine in urine as a diagnostic tool in inherited medium-chain acyl-coenzyme A dehydrogenase deficiency. J Chromatogr B Biomed Sci Appl. 1997 Jun 20;694(1):227-32. [PubMed:9234867 ]
  4. 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 ]
  5. Rinaldo P, O'Shea JJ, Coates PM, Hale DE, Stanley CA, Tanaka K: Medium-chain acyl-CoA dehydrogenase deficiency. Diagnosis by stable-isotope dilution measurement of urinary n-hexanoylglycine and 3-phenylpropionylglycine. N Engl J Med. 1988 Nov 17;319(20):1308-13. [PubMed:3054550 ]
  6. Penzien JM, Molz G, Wiesmann UN, Colombo JP, Buhlmann R, Wermuth B: Medium-chain acyl-CoA dehydrogenase deficiency does not correlate with apparent life-threatening events and the sudden infant death syndrome: results from phenylpropionate loading tests and DNA analysis. Eur J Pediatr. 1994 May;153(5):352-7. [PubMed:8033926 ]


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: