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Record Information
Version4.0
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-10-23 17:28:25 UTC
HMDB IDHMDB0000491
Secondary Accession Numbers
  • HMDB00491
  • HMDB01962
  • HMDB02254
  • HMDB03736
  • HMDB04750
Metabolite Identification
Common Name3-Methyl-2-oxovaleric acid
Description3-Methyl-2-oxovaleric acid is a metabolite of isoleucine in man, animals and bacteria. It is the alpha-keto acid analogue of isoleucine. 3-Methyl-2-oxovaleric acid is produced from isoleucine by cytosolic branched chain aminotransferase 1 (EC:2.6.1.42), whereupon it is further degraded by branched chain keto acid dehydrogenase E1 to 2-Methyl-1-hydroxybutyl-ThPP. It is used as a clinical marker for maple syrup urine disease (MSUD). MSUD is caused by a deficiency of the branched-chain -keto acid dehydrogenase compex resulting in an accumulation of branched-chain amino acids and the corresponding -keto-and -hydroxy acids in blood, urine and cerebrospinal fluid causing neurological damage and mental retardation.
Structure
Thumb
Synonyms
ValueSource
2-oxo-3-Methyl-N-valeric acidChEBI
2-oxo-3-Methylpentanoic acidChEBI
2-oxo-3-MethylvalerateChEBI
2-oxo-3-Methylvaleric acidChEBI
2-OxoisoleucineChEBI
2-Oxokolavenic acidChEBI
3-Ethyl-3-methylpyruvic acidChEBI
3-Methyl-2-oxopentanoic acidChEBI
alpha-keto-beta-Methyl-N-valeric acidChEBI
alpha-keto-beta-Methylvaleric acidChEBI
alpha-oxo-beta-Methyl-N-valeric acidChEBI
alpha-oxo-beta-Methylvaleric acidChEBI
KetoisoleucineChEBI
2-oxo-3-Methyl-N-valerateGenerator
3-Methyl-2-oxovalerateGenerator
2-oxo-3-MethylpentanoateGenerator
2-OxokolavenateGenerator
3-Ethyl-3-methylpyruvateGenerator
3-Methyl-2-oxopentanoateGenerator
a-keto-b-Methyl-N-valerateGenerator
a-keto-b-Methyl-N-valeric acidGenerator
alpha-keto-beta-Methyl-N-valerateGenerator
α-keto-β-methyl-N-valerateGenerator
α-keto-β-methyl-N-valeric acidGenerator
a-keto-b-MethylvalerateGenerator
a-keto-b-Methylvaleric acidGenerator
alpha-keto-beta-MethylvalerateGenerator
α-keto-β-methylvalerateGenerator
α-keto-β-methylvaleric acidGenerator
a-oxo-b-Methyl-N-valerateGenerator
a-oxo-b-Methyl-N-valeric acidGenerator
alpha-oxo-beta-Methyl-N-valerateGenerator
α-oxo-β-methyl-N-valerateGenerator
α-oxo-β-methyl-N-valeric acidGenerator
a-oxo-b-MethylvalerateGenerator
a-oxo-b-Methylvaleric acidGenerator
alpha-oxo-beta-MethylvalerateGenerator
α-oxo-β-methylvalerateGenerator
α-oxo-β-methylvaleric acidGenerator
(3R)-3-Methyl-2-oxopentanoateHMDB
(3R)-3-Methyl-2-oxopentanoic acidHMDB
(R)-3-Methyl-2-oxopentanoateHMDB
(R)-3-Methyl-2-oxopentanoic acidHMDB
(S)-3-Methyl-2-oxopentanoateHMDB
(S)-3-Methyl-2-oxopentanoic acidHMDB
2-keto-3-MethylvalerateHMDB
2-keto-3-Methylvaleric acidHMDB
3-Methyl-2-oxo-pentanoateHMDB
3-Methyl-2-oxo-pentanoic acidHMDB
3-Methyl-2-oxo-valerateHMDB
3-Methyl-2-oxo-valeric acidHMDB
3-Methyl-2-oxovalericHMDB
2-keto MethylvalerateMeSH
KMVAMeSH
alpha-keto-beta-Methylvaleric acid, calcium salt, (S)-isomerMeSH
alpha-keto-beta-Methylvaleric acid, monosodium salt, (S)-isomerMeSH
alpha-keto-beta-Methylvaleric acid, (S)-isomerMeSH
alpha-keto-beta-Methylvaleric acid, calcium saltMeSH
alpha-keto-beta-Methylvaleric acid, sodium salt, (+-)-isomerMeSH
alpha-keto-beta-Methylvaleric acid, (+-)-isomerMeSH
alpha-keto-beta-Methylvaleric acid, sodium saltMeSH
Chemical FormulaC6H10O3
Average Molecular Weight130.1418
Monoisotopic Molecular Weight130.062994186
IUPAC Name3-methyl-2-oxopentanoic acid
Traditional Name3-methyl-2-oxopentanoic acid
CAS Registry Number1460-34-0
SMILES
CCC(C)C(=O)C(O)=O
InChI Identifier
InChI=1S/C6H10O3/c1-3-4(2)5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)
InChI KeyJVQYSWDUAOAHFM-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganic acids and derivatives
Sub ClassKeto acids and derivatives
Direct ParentShort-chain keto acids and derivatives
Alternative Parents
Substituents
  • Branched fatty acid
  • Methyl-branched fatty acid
  • Short-chain keto acid
  • Alpha-keto acid
  • Fatty acyl
  • Alpha-hydroxy ketone
  • Ketone
  • Carboxylic acid derivative
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Carbonyl group
  • Hydrocarbon derivative
  • Organic oxygen compound
  • Organooxygen compound
  • Organic oxide
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

  Health condition:

Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

  Organ and components:

Source:

Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

    Cellular process:

Role

Biological role:

Indirect biological role:

Industrial application:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility915.5 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility9.86 g/LALOGPS
logP1ALOGPS
logP1.75ChemAxon
logS-1.1ALOGPS
pKa (Strongest Acidic)3.52ChemAxon
pKa (Strongest Basic)-9.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity31.79 m³·mol⁻¹ChemAxon
Polarizability13.09 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-000i-9610000000-d2ffd4c2796688c555eeView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-000i-9720000000-9cbc934e100bedd87f17View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-000i-9720000000-5acbdc73aee24c023763View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-000i-6930000000-8c2cf1d8df538010611bView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-000i-6930000000-7bef6751c4c607393e5eView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0hg9-4950000000-3e1c700a6f553bf63afbView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-9610000000-d2ffd4c2796688c555eeView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-9720000000-9cbc934e100bedd87f17View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-9720000000-5acbdc73aee24c023763View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-6930000000-8c2cf1d8df538010611bView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-056r-9000000000-d08073312b337cdae9e6View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-05bo-9400000000-9937bff65302162dc3b9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000w-9300000000-0a6ff60455403c38be39View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0002-9000000000-7ee6d2fb63c6ea4cccfaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-002b-9000000000-47d18dff23b741029581View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-004i-0900000000-ae9da2a9acfa56fc6091View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT , negativesplash10-004r-5900000000-a3f740a5c859439255beView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-06sr-7900000000-4920c1aefcca814ec1f6View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0a4r-9100000000-16995fa8eb603d828a2fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4i-9000000000-a6ccc899039a438533aeView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-004r-5900000000-50107933abbe9c175553View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-01ri-9700000000-23998218af9ee2898594View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9000000000-a30461e2df45eeb8f795View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Location
  • Prostate
Pathways
NameSMPDB/PathwhizKEGG
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase DeficiencyPw000061Pw000061 greyscalePw000061 simpleNot Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencyPw000063Pw000063 greyscalePw000063 simpleNot Available
3-Methylcrotonyl Coa Carboxylase Deficiency Type IPw000065Pw000065 greyscalePw000065 simpleNot Available
3-Methylglutaconic Aciduria Type IPw000066Pw000066 greyscalePw000066 simpleNot Available
3-Methylglutaconic Aciduria Type IIIPw000067Pw000067 greyscalePw000067 simpleNot Available
Displaying entries 1 - 5 of 17 in total
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified18.0 (8.0-31.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified22.7 +/- 4.6 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified2.0 +/- 2.0 uMAdult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
UrineDetected and Quantified0.55-0.95 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified6.005 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified0.53-0.88 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified2.2 (0.4-4.8) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.3 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.24 (0.11 - 0.55) umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified0.54 (0.12 - 1.42) umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected but not Quantified Adult (>18 years old)MaleNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified142.0 +/- 9.0 uMAdult (>18 years old)BothMaple syrup urine disease (MSUD) details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
UrineDetected but not Quantified Adult (>18 years old)Male
Cardiosvacular risk
details
UrineDetected and Quantified4.333 +/- 3.83 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified209.0 +/- 35.0 umol/mmol creatinineAdult (>18 years old)BothMaple syrup urine disease (MSUD) details
Associated Disorders and Diseases
Disease References
Maple syrup urine disease
  1. Schadewaldt P, Hammen HW, Ott AC, Wendel U: Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease. J Inherit Metab Dis. 1999 Aug;22(6):706-22. [PubMed:10472531 ]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB021447
KNApSAcK IDNot Available
Chemspider ID46
KEGG Compound IDC03465
BioCyc ID2-KETO-3-METHYL-VALERATE
BiGG ID35662
Wikipedia LinkNot Available
METLIN ID5478
PubChem Compound47
PDB IDNot Available
ChEBI ID35932
References
Synthesis ReferenceKondo, Shigeo; Sudo, Tetsuji; Ogiwara, Mitsuo; Takeuchi, Hiroshi. a-Oxo-b-methyl-n-valeric acid and its salts. Jpn. Kokai Tokkyo Koho (1979), 3 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
  2. Wendel U, Even G, Langenbeck U, Schadewaldt P, Hummel W: Determination of (S)- and (R)-2-oxo-3-methylvaleric acid in plasma of patients with maple syrup urine disease. Clin Chim Acta. 1992 Jun 15;208(1-2):85-91. [PubMed:1638756 ]
  3. Przyrembel H, Bremer HJ, Duran M, Bruinvis L, Ketting D, Wadman SK, Baumgartner R, Irle U, Bachmann C: Propionyl-CoA carboxylase deficiency with overflow of metabolites of isoleucine catabolism at all levels. Eur J Pediatr. 1979 Jan 18;130(1):1-14. [PubMed:759179 ]

Enzymes

General function:
Involved in catalytic activity
Specific function:
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
BCKDHB
Uniprot ID:
P21953
Molecular weight:
43122.065
Reactions
3-Methyl-2-oxovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxybutyl-ThPP + Carbon dioxidedetails
General function:
Involved in oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
Specific function:
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
BCKDHA
Uniprot ID:
P12694
Molecular weight:
50470.58
Reactions
3-Methyl-2-oxovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxybutyl-ThPP + Carbon dioxidedetails
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
Gene Name:
BCAT1
Uniprot ID:
P54687
Molecular weight:
38644.77
Reactions
L-Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + L-Glutamic aciddetails
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids.
Gene Name:
BCAT2
Uniprot ID:
O15382
Molecular weight:
33776.315
Reactions
L-Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + L-Glutamic aciddetails
General function:
Involved in oxidoreductase activity
Specific function:
Lysosomal L-amino-acid oxidase with highest specific activity with phenylalanine. May play a role in lysosomal antigen processing and presentation (By similarity).
Gene Name:
IL4I1
Uniprot ID:
Q96RQ9
Molecular weight:
65327.26
Reactions
L-Isoleucine + Water + Oxygen → 3-Methyl-2-oxovaleric acid + Ammonia + Hydrogen peroxidedetails