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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:52 UTC
HMDB IDHMDB0000695
Secondary Accession Numbers
  • HMDB0000762
  • HMDB00695
  • HMDB00762
Metabolite Identification
Common NameKetoleucine
DescriptionKetoleucine is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. Ketoleucine is both a neurotoxin and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of ketoleucine are associated with maple syrup urine disease (MSUD). MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD may also present later depending on the severity of the disease. If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465 ).
Structure
Data?1676999704
Synonyms
ValueSource
2-oxo-4-METHYLPENTANOIC ACIDChEBI
2-OxoisocaproateChEBI
4-Methyl-2-oxopentanoateChEBI
4-Methyl-2-oxovaleric acidChEBI
alpha-Ketoisocaproic acidChEBI
2-oxo-4-METHYLPENTANOateGenerator
2-Oxoisocaproic acidGenerator
4-Methyl-2-oxopentanoic acidGenerator
4-Methyl-2-oxovalerateGenerator
a-KetoisocaproateGenerator
a-Ketoisocaproic acidGenerator
alpha-KetoisocaproateGenerator
Α-ketoisocaproateGenerator
Α-ketoisocaproic acidGenerator
2-Keto-4-methylvalerateHMDB
2-Keto-4-methylvaleric acidHMDB
2-KetoisocaproateHMDB
2-Ketoisocaproic acidHMDB
2-oxo-4-MethylvalerateHMDB
2-oxo-4-Methylvaleric acidHMDB
2-OxoleucineHMDB
4-Methyl-2-oxo-valerateHMDB
4-Methyl-2-oxo-valeric acidHMDB
a-KetoisocapronateHMDB
a-Ketoisocapronic acidHMDB
a-OxoisocaproateHMDB
a-Oxoisocaproic acidHMDB
alpha-Keto-isocaproateHMDB
alpha-Keto-isocaproic acidHMDB
alpha-KetoisocapronateHMDB
alpha-Ketoisocapronic acidHMDB
alpha-OxoisocaproateHMDB
alpha-Oxoisocaproic acidHMDB
KetoisocaproateHMDB
Ketoisocaproic acidHMDB
MethyloxovalerateHMDB
Methyloxovaleric acidHMDB
OxoisocaproateHMDB
Oxoisocaproic acidHMDB
Keto-leucineHMDB
Chemical FormulaC6H10O3
Average Molecular Weight130.1418
Monoisotopic Molecular Weight130.062994186
IUPAC Name4-methyl-2-oxopentanoic acid
Traditional Nameketoisocaproate
CAS Registry Number816-66-0
SMILES
CC(C)CC(=O)C(O)=O
InChI Identifier
InChI=1S/C6H10O3/c1-4(2)3-5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)
InChI KeyBKAJNAXTPSGJCU-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassKeto acids and derivatives
Sub ClassShort-chain keto 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
Disposition
Biological locationSource
Process
Role
Physical Properties
StateLiquid
Experimental Molecular Properties
PropertyValueReference
Melting Point8 - 10 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility32 mg/mLNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Astarita_neg125.330932474
Predicted Molecular Properties
PropertyValueSource
Water Solubility6.76 g/LALOGPS
logP0.82ALOGPS
logP1.5ChemAxon
logS-1.3ALOGPS
pKa (Strongest Acidic)3.53ChemAxon
pKa (Strongest Basic)-9.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity31.77 m³·mol⁻¹ChemAxon
Polarizability13 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+129.4831661259
DarkChem[M-H]-124.91631661259
AllCCS[M+H]+130.03532859911
AllCCS[M-H]-128.0232859911
DeepCCS[M+H]+133.60230932474
DeepCCS[M-H]-130.83830932474
DeepCCS[M-2H]-167.44430932474
DeepCCS[M+Na]+142.23930932474
AllCCS[M+H]+130.032859911
AllCCS[M+H-H2O]+125.932859911
AllCCS[M+NH4]+133.932859911
AllCCS[M+Na]+135.032859911
AllCCS[M-H]-128.032859911
AllCCS[M+Na-2H]-130.832859911
AllCCS[M+HCOO]-133.832859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
KetoleucineCC(C)CC(=O)C(O)=O1824.3Standard polar33892256
KetoleucineCC(C)CC(=O)C(O)=O1402.7Standard non polar33892256
KetoleucineCC(C)CC(=O)C(O)=O987.6Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Ketoleucine,1TMS,isomer #1CC(C)CC(=O)C(=O)O[Si](C)(C)C1148.8Semi standard non polar33892256
Ketoleucine,1TMS,isomer #2CC(C)C=C(O[Si](C)(C)C)C(=O)O1235.4Semi standard non polar33892256
Ketoleucine,2TMS,isomer #1CC(C)C=C(O[Si](C)(C)C)C(=O)O[Si](C)(C)C1276.7Semi standard non polar33892256
Ketoleucine,2TMS,isomer #1CC(C)C=C(O[Si](C)(C)C)C(=O)O[Si](C)(C)C1255.8Standard non polar33892256
Ketoleucine,2TMS,isomer #1CC(C)C=C(O[Si](C)(C)C)C(=O)O[Si](C)(C)C1283.4Standard polar33892256
Ketoleucine,1TBDMS,isomer #1CC(C)CC(=O)C(=O)O[Si](C)(C)C(C)(C)C1369.9Semi standard non polar33892256
Ketoleucine,1TBDMS,isomer #2CC(C)C=C(O[Si](C)(C)C(C)(C)C)C(=O)O1468.8Semi standard non polar33892256
Ketoleucine,2TBDMS,isomer #1CC(C)C=C(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1709.4Semi standard non polar33892256
Ketoleucine,2TBDMS,isomer #1CC(C)C=C(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1656.5Standard non polar33892256
Ketoleucine,2TBDMS,isomer #1CC(C)C=C(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1617.1Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-000i-9400000000-49d055f04bfe1877ac5b2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-029j-8930000000-c6c54bf6ee2995a2195b2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0002-1910000000-0ba38257e258733128092014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-MS (2 TMS)splash10-0a5c-4930000000-61247369a805fa2a77a72014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-MS (1 MEOX; 1 TMS)splash10-000i-9510000000-8bfc5a54e29f65edf3612014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-MS (1 MEOX; 1 TMS)splash10-00lj-9620000000-aa8409ceb2359d903f382014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-EI-TOF (Non-derivatized)splash10-000i-9400000000-49d055f04bfe1877ac5b2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-EI-TOF (Non-derivatized)splash10-029j-8930000000-c6c54bf6ee2995a2195b2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-EI-TOF (Non-derivatized)splash10-0002-1910000000-0ba38257e258733128092017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-MS (Non-derivatized)splash10-0a5c-4930000000-61247369a805fa2a77a72017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-MS (Non-derivatized)splash10-000i-9510000000-8bfc5a54e29f65edf3612017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-MS (Non-derivatized)splash10-00lj-9620000000-aa8409ceb2359d903f382017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-EI-TOF (Non-derivatized)splash10-00ks-7930000000-4e9cfd715fa884d30e7f2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Ketoleucine GC-EI-TOF (Non-derivatized)splash10-000i-9500000000-709ebcb57f006703c2692017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Ketoleucine GC-MS (Non-derivatized) - 70eV, Positivesplash10-0006-9000000000-e410bce23706f826f8dd2016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Ketoleucine GC-MS (1 TMS) - 70eV, Positivesplash10-0006-9400000000-c4f06cea1e27a2b246602017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Ketoleucine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Ketoleucine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Ketoleucine GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Ketoleucine GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Ketoleucine GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-0006-9000000000-bed7ac074a60210971e32012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-014i-9200000000-2fa08d2b4df406cf63ea2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-014j-9000000000-045be8e8dca3f93d45ba2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative-QTOFsplash10-004i-0900000000-237fa8ad6bfb929be31e2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative-QTOFsplash10-004r-9700000000-8d38d24fce78b1ff13e62012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative-QTOFsplash10-0a59-9000000000-759cfc0817917283d1a52012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative-QTOFsplash10-0a4l-9000000000-2a068ada71fbe0a5a9882012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative-QTOFsplash10-0a4l-9000000000-58e61e31f3c96d84d7992012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-QQ , negative-QTOFsplash10-004i-0900000000-237fa8ad6bfb929be31e2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-QQ , negative-QTOFsplash10-004r-9700000000-8d38d24fce78b1ff13e62017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-QQ , negative-QTOFsplash10-0a59-9000000000-759cfc0817917283d1a52017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-QQ , negative-QTOFsplash10-0a4l-9000000000-2a068ada71fbe0a5a9882017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-QQ , negative-QTOFsplash10-0a4l-9000000000-58e61e31f3c96d84d7992017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-ITFT , negative-QTOFsplash10-004i-0900000000-556a0d1cf568609752d12017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine LC-ESI-IT , negative-QTOFsplash10-004r-5900000000-20f6923c8ed45e9fa0882017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine 35V, Negative-QTOFsplash10-004i-0900000000-b66d521478c92e2d77d02021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine 10V, Negative-QTOFsplash10-004i-0900000000-9c3a698d9f28af574ef62021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine 40V, Negative-QTOFsplash10-03di-9000000000-47ced70de4860e9002832021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Ketoleucine 20V, Negative-QTOFsplash10-03xr-7900000000-0461a5a56e36a03fe5222021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ketoleucine 10V, Positive-QTOFsplash10-08gr-7900000000-31021fda89eb6535185f2015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ketoleucine 20V, Positive-QTOFsplash10-0btm-9200000000-3bf5df4b557ef87267f42015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ketoleucine 40V, Positive-QTOFsplash10-0a4i-9000000000-37839bf70647d24b94a42015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ketoleucine 10V, Negative-QTOFsplash10-004i-4900000000-796467909d1d4c087a072015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ketoleucine 20V, Negative-QTOFsplash10-01ri-9400000000-0abcd021721f32c10e422015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Ketoleucine 40V, Negative-QTOFsplash10-015l-9000000000-5c33a47dfeb09a1829832015-05-27Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)2012-12-04Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum

IR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)2023-02-03FELIX labView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Neuron
  • Placenta
  • Prostate
  • Skeletal Muscle
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified28.0 (0.0-58.0) uMAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Female
Normal
details
BloodDetected and Quantified33.5 +/- 8.2 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified25-28 uMAdolescent (13-18 years old)FemaleNormal details
BloodDetected and Quantified18 uMAdult (>18 years old)FemaleNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified5.0 +/- 4.4 uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
SalivaDetected and Quantified0.944 +/- 0.442 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified1.24 +/- 0.728 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
UrineDetected and Quantified0-2 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified0.48-0.86 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified1.623 +/- 1.21 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.48-0.72 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0-0.1 umol/mmol creatinineInfant (0-1 year old)Female
Normal
details
UrineDetected and Quantified0-0.2 umol/mmol creatinineInfant (0-1 year old)Male
Normal
details
UrineDetected and Quantified0-0.7 umol/mmol creatinineInfant (0-1 year old)Female
Normal
details
UrineDetected and Quantified0.58 (0.39-0.87) umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • Analysis of 40 NI...
details
UrineDetected and Quantified0.13 (0.02-0.45) umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0.13 (0.03-0.34) umol/mmol creatinineAdult (>18 years old)FemaleNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1918 uMChildren (1-13 years old)Female
Maple syrup urine disease
    • Peritoneal dialys...
details
BloodDetected and Quantified0 uMAdult (>18 years old)FemaleBranched-chain Keto Acid Dehydrogenase Kinase Deficiency details
BloodDetected and Quantified0 uMAdolescent (13-18 years old)FemaleBranched-chain Keto Acid Dehydrogenase Kinase Deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified483 uMChildren (1-13 years old)Female
Maple syrup urine disease
    • Peritoneal dialys...
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleGout details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Femaleankylosing spondylitis details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Femalerheumatoid arthritis details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleAttachment loss  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleMissing teeth details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MalePeriodontal Probing Depth details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleTooth Decay details
UrineDetected and Quantified1.407 +/- 0.928 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
Associated Disorders and Diseases
Disease References
Maple syrup urine disease
  1. Wendel, U., Becker, K., Przyrembel, H. et al. (1980). Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404. Eur J Pediatr.
Branched-chain Keto Acid Dehydrogenase Kinase Deficiency
  1. Novarino G, El-Fishawy P, Kayserili H, Meguid NA, Scott EM, Schroth J, Silhavy JL, Kara M, Khalil RO, Ben-Omran T, Ercan-Sencicek AG, Hashish AF, Sanders SJ, Gupta AR, Hashem HS, Matern D, Gabriel S, Sweetman L, Rahimi Y, Harris RA, State MW, Gleeson JG: Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy. Science. 2012 Oct 19;338(6105):394-7. doi: 10.1126/science.1224631. Epub 2012 Sep 6. [PubMed:22956686 ]
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  2. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  3. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Gout
  1. Shao T, Shao L, Li H, Xie Z, He Z, Wen C: Combined Signature of the Fecal Microbiome and Metabolome in Patients with Gout. Front Microbiol. 2017 Feb 21;8:268. doi: 10.3389/fmicb.2017.00268. eCollection 2017. [PubMed:28270806 ]
Rheumatoid arthritis
  1. Tie-juan ShaoZhi-xing HeZhi-jun XieHai-chang LiMei-jiao WangCheng-ping Wen. Characterization of ankylosing spondylitis and rheumatoid arthritis using 1H NMR-based metabolomics of human fecal extracts. Metabolomics. April 2016, 12:70 [Link]
Attachment loss
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Missing teeth
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Periodontal Probing Depth
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Tooth Decay
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
DrugBank IDDB03229
Phenol Explorer Compound IDNot Available
FooDB IDFDB030510
KNApSAcK IDC00019677
Chemspider ID69
KEGG Compound IDC00233
BioCyc ID2K-4CH3-PENTANOATE
BiGG ID34334
Wikipedia LinkNot Available
METLIN ID5663
PubChem Compound70
PDB IDNot Available
ChEBI ID48430
Food Biomarker OntologyNot Available
VMH ID4MOP
MarkerDB IDMDB00000223
Good Scents IDNot Available
References
Synthesis ReferenceTabuchi, Takeshi; Abe, Matazo. Fermentative preparation of a-ketoisocaproic acid. Jpn. Tokkyo Koho (1970), 3 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Martin PM, Gopal E, Ananth S, Zhuang L, Itagaki S, Prasad BM, Smith SB, Prasad PD, Ganapathy V: Identity of SMCT1 (SLC5A8) as a neuron-specific Na+-coupled transporter for active uptake of L-lactate and ketone bodies in the brain. J Neurochem. 2006 Jul;98(1):279-88. [PubMed:16805814 ]
  2. 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 ]
  3. Yudkoff M, Daikhin Y, Nissim I, Horyn O, Luhovyy B, Lazarow A, Nissim I: Brain amino acid requirements and toxicity: the example of leucine. J Nutr. 2005 Jun;135(6 Suppl):1531S-8S. [PubMed:15930465 ]
  4. Chow LS, Albright RC, Bigelow ML, Toffolo G, Cobelli C, Nair KS: Mechanism of insulin's anabolic effect on muscle: measurements of muscle protein synthesis and breakdown using aminoacyl-tRNA and other surrogate measures. Am J Physiol Endocrinol Metab. 2006 Oct;291(4):E729-36. Epub 2006 May 16. [PubMed:16705065 ]
  5. Wang Y, Holmes E, Nicholson JK, Cloarec O, Chollet J, Tanner M, Singer BH, Utzinger J: Metabonomic investigations in mice infected with Schistosoma mansoni: an approach for biomarker identification. Proc Natl Acad Sci U S A. 2004 Aug 24;101(34):12676-81. Epub 2004 Aug 16. [PubMed:15314235 ]
  6. Mitch WE, Walser M, Sapir DG: Nitrogen sparing induced by leucine compared with that induced by its keto analogue, alpha-ketoisocaproate, in fasting obese man. J Clin Invest. 1981 Feb;67(2):553-62. [PubMed:7462428 ]
  7. Sgaravatti AM, Rosa RB, Schuck PF, Ribeiro CA, Wannmacher CM, Wyse AT, Dutra-Filho CS, Wajner M: Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease. Biochim Biophys Acta. 2003 Nov 20;1639(3):232-8. [PubMed:14636955 ]
  8. 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 ]
  9. Hachey DL, Patterson BW, Reeds PJ, Elsas LJ: Isotopic determination of organic keto acid pentafluorobenzyl esters in biological fluids by negative chemical ionization gas chromatography/mass spectrometry. Anal Chem. 1991 May 1;63(9):919-23. [PubMed:1858984 ]
  10. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

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
Ketoleucine + Thiamine pyrophosphate → 3-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
Ketoleucine + Thiamine pyrophosphate → 3-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-Leucine + Oxoglutaric acid → Ketoleucine + 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-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic aciddetails