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
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-03-02 21:25:54 UTC
HMDB IDHMDB00459
Secondary Accession NumbersNone
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 2.3.1.13) 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 ).
Structure
Thumb
Synonyms
ValueSource
beta-MethylcrotonylglycineChEBI
b-MethylcrotonylglycineGenerator
β-methylcrotonylglycineGenerator
3-Methylcrotonyl glycineHMDB
3-Methylcrotonyl-glycineHMDB
3-MethylcrotonylglycinHMDB
N-(3-Methyl-1-oxo-2-butenyl)-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
SMILES
CC(C)=CC(=O)NCC(O)=O
InChI Identifier
InChI=1S/C7H11NO3/c1-5(2)3-6(9)8-4-7(10)11/h3H,4H2,1-2H3,(H,8,9)(H,10,11)
InChI KeyPFWQSHXPNKRLIV-UHFFFAOYSA-N
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
Substituents
  • 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
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locations
  • Cytoplasm
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility7.64 mg/mLALOGPS
logP0.02ALOGPS
logP0.058ChemAxon
logS-1.3ALOGPS
pKa (Strongest Acidic)4.09ChemAxon
pKa (Strongest Basic)1.01ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area66.4 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity40.05 m3·mol-1ChemAxon
Polarizability15.79 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-001i-9000000000-de6b64abad08a6b0aa93View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0a59-9000000000-6614867cbb98a3422626View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0a59-9000000000-87fe2c98c5568e0a3651View in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
Biofluid Locations
  • Blood
  • Urine
Tissue Location
  • Fibroblasts
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not Quantified Not AvailableNot Available
Normal
    details
    UrineDetected and Quantified1.0 (0.0-2.0) 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<10 umol/mmol creatinineChildren (1 - 18 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0.8-1.2 umol/mmol creatinineAdult (>18 years old)MaleNormal details
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReferenceDetails
    UrineDetected and Quantified700.0 (400.0-1000.0) umol/mmol creatinineChildren (1-13 years old)Both3-Methyl-Crotonyl-Glycinuria details
    UrineDetected and Quantified27.5 (5.0-50.0) umol/mmol creatinineChildren (1-13 years old)BothBiotinidase deficiency details
    UrineDetected and Quantified225.0 (0.00-450.0) umol/mmol creatinineChildren (1-13 years old)Both3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
    Associated Disorders and Diseases
    Disease References
    3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
    1. MetaGene [Link]
    Biotinidase deficiency
    1. MetaGene [Link]
    Associated OMIM IDs
    • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
    • 253260 (Biotinidase deficiency)
    DrugBank IDNot Available
    DrugBank Metabolite IDNot Available
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB IDFDB022057
    KNApSAcK IDNot Available
    Chemspider ID148224
    KEGG Compound IDNot Available
    BioCyc IDNot Available
    BiGG IDNot Available
    Wikipedia LinkNot Available
    NuGOwiki LinkHMDB00459
    Metagene LinkHMDB00459
    METLIN ID5447
    PubChem Compound169485
    PDB IDNot Available
    ChEBI IDNot Available
    References
    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. 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 ]
    2. 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 ]
    3. 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 ]
    4. 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 ]
    5. 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 ]
    6. 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 ]

    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