You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-02-27 20:29:57 UTC
Secondary Accession Numbers
  • HMDB00459
Metabolite Identification
Common Name3-Methylcrotonylglycine
Description3-Methylcrotonylglycine 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-acylglycine. 3-Methylcrotonylglycine is a normal amino acid metabolite found in urine. Increased levels of this metabolite are found in patients suffering from leucine catabolic disorders, such as 3-methylcrotonyl-CoA carboxylase deficiency. 3-Methylcrotonylglycine is often considered to be a diagnostic marker of organic acidemias (PMID 11170888 ). Moreover, 3-methylcrotonylglycine is found to be associated with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency and propionic acidemia, which are also inborn errors of metabolism.
3-Methylcrotonyl glycineHMDB
Chemical FormulaC7H11NO3
Average Molecular Weight157.1671
Monoisotopic Molecular Weight157.073893223
IUPAC Name2-(3-methylbut-2-enamido)acetic acid
Traditional Name(3-methylbut-2-enamido)acetic acid
CAS Registry Number33008-07-0
InChI Identifier
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
  • N-acyl-alpha-amino acid
  • N-acyl-amine
  • Secondary carboxylic acid amide
  • Carboxamide group
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors


Biological location:


Biological role:

Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility7.64 g/LALOGPS
pKa (Strongest Acidic)4.09ChemAxon
pKa (Strongest Basic)-0.54ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area66.4 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity40.05 m³·mol⁻¹ChemAxon
Polarizability15.79 ųChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-001l-9200000000-b5de5c506d0864c0f7d1Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-01x3-9210000000-3cd3c3c8ea08c7d1e6c7Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-001i-9000000000-de6b64abad08a6b0aa93Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0a59-9000000000-6614867cbb98a3422626Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0a59-9000000000-87fe2c98c5568e0a3651Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0a4i-7900000000-f113c2d59fe1238ea573Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0a6r-9100000000-ca7eb3d4b533d2e31892Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4i-9000000000-78c1b06293f14b96b012Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0a4i-0900000000-9462ca1f1e0bcf30b871Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0a4i-6900000000-7cf2a248f3b7490e7677Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0ab9-9000000000-30da48e2c1ce30e69097Spectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Biological Properties
Cellular Locations
  • Cytoplasm
Biospecimen Locations
  • Blood
  • Urine
Tissue Locations
  • Fibroblasts
Normal Concentrations
BloodExpected but not Quantified Not AvailableNot Available
    UrineDetected and Quantified0.0559–0.6455 umol/mmol creatinineAdult (25-30 years old)Both
    Not Available
    UrineDetected and Quantified0-2 umol/mmol creatinineNewborn (0-30 days old)Both
    UrineDetected and Quantified0.040.0–0.6236 umol/mmol creatinineAdult (25-30 years old)Both
    Not Available
    UrineDetected and Quantified0.0651–0.4442 umol/mmol creatinineAdult (25-30 years old)Both
    Not Available
    UrineDetected but not Quantified Adult (24-38years old)Not SpecifiedNormal details
    UrineDetected and Quantified<0.48 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified0.9-2.0 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
    UrineDetected and Quantified<2 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
    UrineDetected and Quantified<10 umol/mmol creatinineChildren (1 - 18 years old)Both
      • BC Children's Hos...
    UrineDetected and Quantified6.100 +/- 4.100 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal
      • Geigy Scientific ...
    UrineDetected and Quantified0.8-1.2 umol/mmol creatinineAdult (>18 years old)MaleNormal details
    Abnormal Concentrations
    UrineDetected and Quantified700.0 (400.0-1000.0) umol/mmol creatinineChildren (1-13 years old)Both3-Methyl-Crotonyl-Glycinuria
      • MetaGene: Metabol...
    UrineDetected and Quantified27.5 (5.0-50.0) umol/mmol creatinineChildren (1-13 years old)BothBiotinidase deficiency
      • MetaGene: Metabol...
    UrineDetected and Quantified0.41 umol/mmol creatinineAdult (>18 years old)Not Specified
    Propionic acidemia
    UrineDetected and Quantified27.6 umol/mmol creatinineNewborn (0-30 days old)Male3-hydroxy-3-methylglutaric- coenzyme A lyase deficiency details
    UrineDetected and Quantified120 umol/mmol creatinineChildren (1 - 13 years old)Female3-Methylcrotonyl-CoA Carboxylase Deficiency details
    UrineDetected and Quantified1975 umol/mmol creatinineChildren (1 - 13 years old)Not SpecifiedIsolated 3-Methylcrotonyl-CoA Carboxylase Deficiency details
    UrineDetected and Quantified27.6 umol/mmol creatinineNewborn (0-30 days old)Male3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
    UrineDetected and Quantified225.0 (0.00-450.0) umol/mmol creatinineChildren (1-13 years old)Both3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
      • MetaGene: Metabol...
    Associated Disorders and Diseases
    Disease References
    3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
    1. Santarelli F, Cassanello M, Enea A, Poma F, D'Onofrio V, Guala G, Garrone G, Puccinelli P, Caruso U, Porta F, Spada M: A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Ital J Pediatr. 2013 May 24;39:33. doi: 10.1186/1824-7288-39-33. [PubMed:23705938 ]
    2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: METAGENE consortium.
    1. Rutledge SL, Berry GT, Stanley CA, van Hove JL, Millington D: Glycine and L-carnitine therapy in 3-methylcrotonyl-CoA carboxylase deficiency. J Inherit Metab Dis. 1995;18(3):299-305. [PubMed:7474896 ]
    2. de Kremer RD, Latini A, Suormala T, Baumgartner ER, Larovere L, Civallero G, Guelbert N, Paschini-Capra A, Depetris-Boldini C, Mayor CQ: Leukodystrophy and CSF purine abnormalities associated with isolated 3-methylcrotonyl-CoA carboxylase deficiency. Metab Brain Dis. 2002 Mar;17(1):13-8. [PubMed:11893004 ]
    3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: METAGENE consortium.
    Biotinidase deficiency
    1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: 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
    • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
    • 210200 (3-Methyl-crotonyl-glycinuria)
    • 253260 (Biotinidase deficiency)
    • 606054 (Propionic acidemia)
    DrugBank IDNot Available
    Phenol Explorer Compound IDNot Available
    FooDB IDFDB022057
    KNApSAcK IDNot Available
    Chemspider ID148224
    KEGG Compound IDNot Available
    BioCyc IDNot Available
    BiGG IDNot Available
    Wikipedia LinkNot Available
    METLIN ID5447
    PubChem Compound169485
    PDB IDNot Available
    ChEBI ID68499
    Food Biomarker OntologyNot Available
    VMH IDNot Available
    Synthesis ReferenceCarter, S. M. Bonham; Watson, D. G.; Midgley, J. M.; Logan, R. W. Synthesis and characterization of acyl glycines. Their measurement in single blood spots by gas chromatography-mass spectrometry to diagnose inborn errors of metabolism. Journal of Chromatography, B: Biomedical Applications (1996), 677(1), 29-35.
    Material Safety Data Sheet (MSDS)Not Available
    General References
    1. Wysocki SJ, Hahnel R: 3-Methylcrotonylglycine excretion in 3-hydroxy-3-methylglutaric aciduria. Clin Chim Acta. 1978 May 16;86(1):101-8. [PubMed:657530 ]
    2. Koeberl DD, Millington DS, Smith WE, Weavil SD, Muenzer J, McCandless SE, Kishnani PS, McDonald MT, Chaing S, Boney A, Moore E, Frazier DM: Evaluation of 3-methylcrotonyl-CoA carboxylase deficiency detected by tandem mass spectrometry newborn screening. J Inherit Metab Dis. 2003;26(1):25-35. [PubMed:12872837 ]
    3. Baykal T, Gokcay GH, Ince Z, Dantas MF, Fowler B, Baumgartner MR, Demir F, Can G, Demirkol M: Consanguineous 3-methylcrotonyl-CoA carboxylase deficiency: early-onset necrotizing encephalopathy with lethal outcome. J Inherit Metab Dis. 2005;28(2):229-33. [PubMed:15877210 ]
    4. Cowan MJ, Wara DW, Packman S, Ammann AJ, Yoshino M, Sweetman L, Nyhan W: Multiple biotin-dependent carboxylase deficiencies associated with defects in T-cell and B-cell immunity. Lancet. 1979 Jul 21;2(8134):115-8. [PubMed:88554 ]
    5. Gallardo ME, Desviat LR, Rodriguez JM, Esparza-Gordillo J, Perez-Cerda C, Perez B, Rodriguez-Pombo P, Criado O, Sanz R, Morton DH, Gibson KM, Le TP, Ribes A, de Cordoba SR, Ugarte M, Penalva MA: The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolism. Am J Hum Genet. 2001 Feb;68(2):334-46. Epub 2001 Jan 17. [PubMed:11170888 ]
    6. Chantin C, Bonin B, Boulieu R, Bory C: Liquid-chromatographic study of purine metabolism abnormalities in purine nucleoside phosphorylase deficiency. Clin Chem. 1996 Feb;42(2):326-8. [PubMed:8595732 ]


    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: