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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:03:11 UTC
HMDB IDHMDB00678
Secondary Accession NumbersNone
Metabolite Identification
Common NameIsovalerylglycine
DescriptionIsovalerylglycine 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. Isovalerylglycine is a byproduct of the catabolism of the aminoacid leucine. Accumulation of isovalerylglycine occurs in Isovaleric Acidemia (IVA). IVA (OMIM/ McKusick 243500) is an autosomal recessive disorder caused by mutations in the isovaleryl-CoA dehydrogenase (EC 1.3.99.10) gene. The deficiency of this enzyme in the metabolism of leucine leads to the accumulation of a series of isovaleryl-CoA metabolites, such as isovalerylglycine (IVG). It is very important to caution for false positive results when screening for isovaleric acidemia by tandem mass spectrometry based on dried blood-spot levels of C5-acylcarnitines, including isovalerylcarnitine and its isomer, pivaloylcarnitine; pivaloylcarnitine is derived from pivalate-generating antibiotics, and has caused many false-positive results. (PMID: 17850781 ).
Structure
Thumb
Synonyms
ValueSource
2-(3-methylbutanoylamino)Ethanoic acidChEBI
IsopentanoylglycineChEBI
IsovalerylglycineChEBI
N-IsopentanoylglycineChEBI
N-IsovaleroylglycineChEBI
2-(3-methylbutanoylamino)EthanoateGenerator
2-(3-methylbutanoylamino)AcetateGenerator
3-MethylbutyrylglycineHMDB
N-Isovaleryl-glycineHMDB
[(3-Methylbutanoyl)amino]acetateHMDB
[(3-Methylbutanoyl)amino]acetic acidHMDB
Chemical FormulaC7H13NO3
Average Molecular Weight159.183
Monoisotopic Molecular Weight159.089543287
IUPAC Name2-(3-methylbutanamido)acetic acid
Traditional Name(3-methylbutanamido)acetic acid
CAS Registry Number16284-60-9
SMILES
CC(C)CC(=O)NCC(O)=O
InChI Identifier
InChI=1S/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11)
InChI KeyInChIKey=ZRQXMKMBBMNNQC-UHFFFAOYSA-N
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
Substituents
  • N-acyl-aliphatic-alpha amino acid
  • Fatty acyl
  • N-acyl-amine
  • Fatty amide
  • Secondary carboxylic acid amide
  • Carboxamide group
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid amide
  • 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 locationsNot Available
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point87 - 90 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility6.5 mg/mLALOGPS
logP0.35ALOGPS
logP0.1ChemAxon
logS-1.4ALOGPS
pKa (Strongest Acidic)4.17ChemAxon
pKa (Strongest Basic)-1.1ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area66.4 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity39.22 m3·mol-1ChemAxon
Polarizability16.39 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)splash10-004r-9000000000-0a3d8ca5efe599736988View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0a4i-9000000000-bdd258e50d1f36946722View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-052f-9000000000-c69489d7cef77e37b417View 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
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.17 +/- 0.03 uMNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified3.8 +/- 0.9 umol/mmol creatinineAdolescent (13-18 years old)FemaleNormal details
UrineDetected and Quantified2.0 (0.4-4.0) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.37 +/- 1.19 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified2.322 +/- 1.914 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified<10 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified40.0 (1.3-80.0) uMNewborn (0-30 days old)BothIsovaleric acidemia details
UrineDetected and Quantified7.7 +/- 4.8 umol/mmol creatinineAdolescent (13-18 years old)FemaleAnorexia nervosa details
UrineDetected and Quantified2.813 +/- 2.181 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified5.5 (1.0-10.0) umol/mmol creatinineAdult (>18 years old)BothEthylmalonic encephalopathy details
UrineDetected and Quantified110.0 (20.0-200.0) umol/mmol creatinineChildren (1-13 years old)BothEthylmalonic encephalopathy details
UrineDetected and Quantified500.00(2.00-1000.00) umol/mmol creatinineChildren (1-13 years old)BothGlutaric Aciduria II details
UrineDetected and Quantified5500.00 (2000.00-9000.000 umol/mmol creatinineChildren (1-13 years old)BothIsovaleric acidemia details
Associated Disorders and Diseases
Disease References
Anorexia nervosa
  1. Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [10197568 ]
Glutaric acidemia type 2
  1. MetaGene [Link]
Isovaleric acidemia
  1. Shigematsu Y, Hata I, Tanaka Y: Stable-isotope dilution measurement of isovalerylglycine by tandem mass spectrometry in newborn screening for isovaleric acidemia. Clin Chim Acta. 2007 Nov-Dec;386(1-2):82-6. Epub 2007 Aug 19. [17850781 ]
  2. MetaGene [Link]
Ethylmalonic encephalopathy
  1. MetaGene [Link]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022177
KNApSAcK IDNot Available
Chemspider ID475516
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00678
Metagene LinkHMDB00678
METLIN ID5647
PubChem Compound546304
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceBondi, S.; Eissler, F. Lipoproteins and the Fatty Degeneration of Cells. Biochemische Zeitschrift (1910), 23 499-513.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Fries MH, Rinaldo P, Schmidt-Sommerfeld E, Jurecki E, Packman S: Isovaleric acidemia: response to a leucine load after three weeks of supplementation with glycine, L-carnitine, and combined glycine-carnitine therapy. J Pediatr. 1996 Sep;129(3):449-52. [8804338 ]
  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. [7360517 ]
  3. Hine DG, Hack AM, Goodman SI, Tanaka K: Stable isotope dilution analysis of isovalerylglycine in amniotic fluid and urine and its application for the prenatal diagnosis of isovaleric acidemia. Pediatr Res. 1986 Mar;20(3):222-6. [3703611 ]
  4. Shigematsu Y, Hata I, Tanaka Y: Stable-isotope dilution measurement of isovalerylglycine by tandem mass spectrometry in newborn screening for isovaleric acidemia. Clin Chim Acta. 2007 Nov-Dec;386(1-2):82-6. Epub 2007 Aug 19. [17850781 ]

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