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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-10-23 18:17:41 UTC
HMDB IDHMDB0000730
Secondary Accession Numbers
  • HMDB00730
Metabolite Identification
Common NameIsobutyrylglycine
DescriptionIsobutyrylglycine 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-acylglycineIsobutyrylglycine is identified in large amount in urine of patients with isobutyryl-CoA dehydrogenase deficiency. Isobutyryl-CoA dehydrogenase deficiency is a disorder caused by the deficiency of isobutyryl-CoA dehydrogenase that is involved in the catabolism of the branched-chain amino acid valine (PMID 15505379 ). Isobutyrylglycine is a biomarker for the consumption of cheese.
Structure
Thumb
Synonyms
ValueSource
IsobutanoylglycineChEBI
N-IsobutanoylglycineChEBI
N-(1-Methylpropyl)glycineMeSH
N-IsobutyrylglycineMeSH
Chemical FormulaC6H11NO3
Average Molecular Weight145.1564
Monoisotopic Molecular Weight145.073893223
IUPAC Name2-(2-methylpropanamido)acetic acid
Traditional Nameisobutyrylglycine
CAS Registry Number15926-18-8
SMILES
CC(C)C(=O)NCC(O)=O
InChI Identifier
InChI=1S/C6H11NO3/c1-4(2)6(10)7-3-5(8)9/h4H,3H2,1-2H3,(H,7,10)(H,8,9)
InChI KeyDCICDMMXFIELDF-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
  • 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
Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

Source:

Role

Indirect biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point82.5 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility17.9 g/LALOGPS
logP-0.11ALOGPS
logP-0.085ChemAxon
logS-0.91ALOGPS
pKa (Strongest Acidic)4.06ChemAxon
pKa (Strongest Basic)-1.2ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area66.4 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity34.65 m³·mol⁻¹ChemAxon
Polarizability14.56 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-00dl-9100000000-91e12bffc069cc1fe9a0View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00di-9100000000-5281458abfc09bd42ce1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-00fr-9000000000-f1d95bb7599bb8f6cd73View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0006-9000000000-b41069b8dbfe4b85b346View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0006-9000000000-85cb61c87f73b734a9e5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-006t-4900000000-56e60896238c44e6c163View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00dl-9300000000-b49a0fcfcc97adc64a23View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0596-9000000000-19f4499051d30aa160e8View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0006-0900000000-96c02d5f830c0ad4ba52View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0f6x-1900000000-be6409aa473bdd7dc417View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-05fr-9000000000-2656458192decd1b1fbfView in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Urine
Tissue LocationNot Available
PathwaysNot Available
NameSMPDB/PathwhizKEGG
No entries found
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected but not Quantified Adult (24-38years old)Not SpecifiedNormal details
UrineDetected but not Quantified Adult (>18 years old)BothNormal details
UrineDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified<2.9 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0.2157–1.6217 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
UrineDetected and Quantified0.1742–1.2490 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
UrineDetected and Quantified0.1643–0.8386 umol/mmol creatinineAdult (25-30 years old)Both
Not Available
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified2.15 (0.5-3.8) umol/mmol creatinineAdult (>18 years old)BothEthylmalonic encephalopathy details
UrineDetected and Quantified26.0 (4.0-48.0) umol/mmol creatinineChildren (1-13 years old)BothEthylmalonic encephalopathy details
UrineDetected and Quantified100.00 (0.00-200.00) umol/mmol creatinineChildren (1-13 years old)BothGlutaric Aciduria II details
Associated Disorders and Diseases
Disease References
Glutaric acidemia type 2
    MetaGene
Ethylmalonic encephalopathy
    MetaGene
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022208
KNApSAcK IDNot Available
Chemspider ID9030891
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID5698
PubChem Compound10855600
PDB IDNot Available
ChEBI ID70979
References
Synthesis ReferenceKatz, J.; Lieberman, I.; Barker, H. A. Formation of propionyl-, butyryl-, and other acylglycines by enzymes of Clostridium kluyveri. Journal of Biological Chemistry (1953), 200 431-41.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Sass JO, Sander S, Zschocke J: Isobutyryl-CoA dehydrogenase deficiency: isobutyrylglycinuria and ACAD8 gene mutations in two infants. J Inherit Metab Dis. 2004;27(6):741-5. [PubMed:15505379 ]
  2. Goodman SI, McCabe ER, Fennessey PV, Mace JW: Multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II) with transient hypersarcosinemia and sarcosinuria; possible inherited deficiency of an electron transfer flavoprotein. Pediatr Res. 1980 Jan;14(1):12-7. [PubMed:7360517 ]

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