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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:54 UTC
HMDB IDHMDB0000011
Secondary Accession Numbers
  • HMDB0000357
  • HMDB00011
  • HMDB00357
Metabolite Identification
Common Name3-Hydroxybutyric acid
Description3-Hydroxybutyric acid (CAS: 300-85-6), also known as beta-hydroxybutanoic acid, is a typical partial-degradation product of branched-chain amino acids (primarily valine) released from muscle for hepatic and renal gluconeogenesis. This acid is metabolized by 3-hydroxybutyrate dehydrogenase (catalyzes the oxidation of 3-hydroxybutyrate to form acetoacetate, using NAD+ as an electron acceptor). The enzyme functions in nervous tissues and muscles, enabling the use of circulating hydroxybutyrate as a fuel. In the liver mitochondrial matrix, the enzyme can also catalyze the reverse reaction, a step in ketogenesis. 3-Hydroxybutyric acid is a chiral compound having two enantiomers, D-3-hydroxybutyric acid and L-3-hydroxybutyric acid, and is a ketone body. Like the other ketone bodies (acetoacetate and acetone), levels of 3-hydroxybutyrate in blood and urine are raised in ketosis. In humans, 3-hydroxybutyrate is synthesized in the liver from acetyl-CoA and can be used as an energy source by the brain when blood glucose is low. Blood levels of 3-hydroxybutyric acid levels may be monitored in diabetic patients to look for diabetic ketoacidosis. Persistent mild hyperketonemia is a common finding in newborns. Ketone bodies serve as an indispensable source of energy for extrahepatic tissues, especially the brain and lung of developing mammals. Another important function of ketone bodies is to provide acetoacetyl-CoA and acetyl-CoA for the synthesis of cholesterol, fatty acids, and complex lipids. During the early postnatal period, acetoacetate (AcAc) and beta-hydroxybutyrate are preferred over glucose as substrates for the synthesis of phospholipids and sphingolipids in accord with requirements for brain growth and myelination. Thus, during the first two weeks of postnatal development, when the accumulation of cholesterol and phospholipids accelerates, the proportion of ketone bodies incorporated into these lipids increases. On the other hand, an increased proportion of ketone bodies is utilized for cerebroside synthesis during the period of active myelination. In the lung, AcAc serves better than glucose as a precursor for the synthesis of lung phospholipids. The synthesized lipids, particularly dipalmitoylphosphatidylcholine, are incorporated into surfactant, and thus have a potential role in supplying adequate surfactant lipids to maintain lung function during the early days of life (PMID: 3884391 ). 3-Hydroxybutyric acid is found to be associated with fumarase deficiency and medium-chain acyl-CoA dehydrogenase deficiency, which are inborn errors of metabolism. 3-Hydroxybutyric acid is a metabolite of Alcaligenes and can be produced from plastic metabolization or incorporated into polymers, depending on the species (PMID: 7646009 , 18615882 ).
Structure
Data?1676999667
Synonyms
ValueSource
(R)-(-)-beta-Hydroxybutyric acidChEBI
(R)-3-Hydroxybutanoic acidChEBI
3-D-Hydroxybutyric acidChEBI
D-3-Hydroxybutyric acidChEBI
D-beta-Hydroxybutyric acidKegg
(R)-(-)-b-HydroxybutyrateGenerator
(R)-(-)-b-Hydroxybutyric acidGenerator
(R)-(-)-beta-HydroxybutyrateGenerator
(R)-(-)-Β-hydroxybutyrateGenerator
(R)-(-)-Β-hydroxybutyric acidGenerator
(R)-3-HydroxybutanoateGenerator
3-D-HydroxybutyrateGenerator
D-3-HydroxybutyrateGenerator
D-b-HydroxybutyrateGenerator
D-b-Hydroxybutyric acidGenerator
D-beta-HydroxybutyrateGenerator
D-Β-hydroxybutyrateGenerator
D-Β-hydroxybutyric acidGenerator
(R)-3-HydroxybutyrateGenerator
3-delta-HydroxybutyrateHMDB
3-delta-Hydroxybutyric acidHMDB
BHIBHMDB
D-(-)-3-HydroxybutyrateHMDB
delta-(-)-3-HydroxybutyrateHMDB
delta-3-HydroxybutyrateHMDB
delta-3-Hydroxybutyric acidHMDB
delta-beta-HydroxybutyrateHMDB
3R-Hydroxy-butanoateHMDB
(R)-3-Hydroxybutyric acidKEGG
(-)-3-Hydroxy-n-butyric acidHMDB
(-)-3-Hydroxybutyric acidHMDB
(3R)-3-Hydroxybutanoic acidHMDB
(3R)-3-Hydroxybutyric acidHMDB
(3R)-HydroxybutyrateHMDB
(R)-(-)-3-Hydroxybutyric acidHMDB
(R)-beta-Hydroxybutanoic acidHMDB
(R)-beta-Hydroxybutyric acidHMDB
(R)-β-Hydroxybutanoic acidHMDB
(R)-β-Hydroxybutyric acidHMDB
3-Hydroxy-n-butyric acidHMDB
3-Hydroxybutanoic acidHMDB
3-Hydroxybutyric acidHMDB
3R-Hydroxybutanoic acidHMDB
D-(-)-3-Hydroxybutanoic acidHMDB
D-(-)-3-Hydroxybutyric acidHMDB
D-(-)-beta-Hydroxybutyric acidHMDB
D-(-)-β-Hydroxybutyric acidHMDB
beta-Hydroxy-n-butyric acidHMDB
beta-Hydroxybutanoic acidHMDB
beta-Hydroxybutyric acidHMDB
β-Hydroxy-n-butyric acidHMDB
β-Hydroxybutanoic acidHMDB
β-Hydroxybutyric acidHMDB
Chemical FormulaC4H8O3
Average Molecular Weight104.1045
Monoisotopic Molecular Weight104.047344122
IUPAC Name(3R)-3-hydroxybutanoic acid
Traditional Name(-)-3-hydroxybutyric acid
CAS Registry Number625-72-9
SMILES
C[C@@H](O)CC(O)=O
InChI Identifier
InChI=1S/C4H8O3/c1-3(5)2-4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m1/s1
InChI KeyWHBMMWSBFZVSSR-GSVOUGTGSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as beta hydroxy acids and derivatives. Beta hydroxy acids and derivatives are compounds containing a carboxylic acid substituted with a hydroxyl group on the C3 carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassHydroxy acids and derivatives
Sub ClassBeta hydroxy acids and derivatives
Direct ParentBeta hydroxy acids and derivatives
Alternative Parents
Substituents
  • Short-chain hydroxy acid
  • Beta-hydroxy acid
  • Fatty acid
  • Secondary alcohol
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Disposition
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point49 - 50 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility539 g/LALOGPS
logP-0.5ALOGPS
logP-0.39ChemAxon
logS0.71ALOGPS
pKa (Strongest Acidic)4.41ChemAxon
pKa (Strongest Basic)-2.6ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area57.53 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity23.46 m³·mol⁻¹ChemAxon
Polarizability9.96 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]+121.11631661259
DarkChem[M-H]-117.28531661259
AllCCS[M+H]+126.73432859911
AllCCS[M-H]-124.25732859911
DeepCCS[M+H]+122.28130932474
DeepCCS[M-H]-119.21530932474
DeepCCS[M-2H]-155.68130932474
DeepCCS[M+Na]+130.84230932474
AllCCS[M+H]+126.732859911
AllCCS[M+H-H2O]+122.432859911
AllCCS[M+NH4]+130.832859911
AllCCS[M+Na]+131.932859911
AllCCS[M-H]-124.332859911
AllCCS[M+Na-2H]-128.132859911
AllCCS[M+HCOO]-132.432859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
3-Hydroxybutyric acidC[C@@H](O)CC(O)=O2109.0Standard polar33892256
3-Hydroxybutyric acidC[C@@H](O)CC(O)=O962.0Standard non polar33892256
3-Hydroxybutyric acidC[C@@H](O)CC(O)=O986.1Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
3-Hydroxybutyric acid,1TMS,isomer #1C[C@H](CC(=O)O)O[Si](C)(C)C1074.4Semi standard non polar33892256
3-Hydroxybutyric acid,1TMS,isomer #2C[C@@H](O)CC(=O)O[Si](C)(C)C1018.1Semi standard non polar33892256
3-Hydroxybutyric acid,2TMS,isomer #1C[C@H](CC(=O)O[Si](C)(C)C)O[Si](C)(C)C1168.2Semi standard non polar33892256
3-Hydroxybutyric acid,1TBDMS,isomer #1C[C@H](CC(=O)O)O[Si](C)(C)C(C)(C)C1313.6Semi standard non polar33892256
3-Hydroxybutyric acid,1TBDMS,isomer #2C[C@@H](O)CC(=O)O[Si](C)(C)C(C)(C)C1235.9Semi standard non polar33892256
3-Hydroxybutyric acid,2TBDMS,isomer #1C[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)O[Si](C)(C)C(C)(C)C1603.0Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - 3-Hydroxybutyric acid EI-B (Non-derivatized)splash10-0002-0900000000-7410b547b9d02c6326c72017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 3-Hydroxybutyric acid EI-B (Non-derivatized)splash10-0002-0900000000-7410b547b9d02c6326c72018-05-18HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 3-Hydroxybutyric acid GC-MS (Non-derivatized) - 70eV, Positivesplash10-0007-9000000000-5f169537ace358b06fd02017-09-01Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 3-Hydroxybutyric acid GC-MS (2 TMS) - 70eV, Positivesplash10-01ei-9710000000-2652bd46b41e50defdb02017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 3-Hydroxybutyric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 3-Hydroxybutyric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 3-Hydroxybutyric acid GC-MS (TMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 3-Hydroxybutyric acid GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 3-Hydroxybutyric acid GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 3-Hydroxybutyric acid GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 3-Hydroxybutyric acid GC-MS (TBDMS_2_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
MSMass Spectrum (Electron Ionization)splash10-0007-9000000000-7fc414806153ebd8822c2020-03-25Not AvailableView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid Quattro_QQQ 10V, Negative-QTOF (Annotated)splash10-0a4i-9200000000-4156904e7472b5e972492012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid Quattro_QQQ 25V, Negative-QTOF (Annotated)splash10-0a4i-9300000000-505ae46abb0c49c78b1e2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid Quattro_QQQ 40V, Negative-QTOF (Annotated)splash10-0zfr-9600000000-dfed69c37c1a4d7944402012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative-QTOFsplash10-0udi-1900000000-b34932b6f2ee5bca96972012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative-QTOFsplash10-0a4i-9100000000-cdbb3586477ed705b4d72012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative-QTOFsplash10-0a4i-9000000000-022ab74e163dd752d0542012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative-QTOFsplash10-0006-9000000000-18a18150fcc1dfee58cf2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative-QTOFsplash10-0006-9000000000-e8d8003bcda8aa818d4e2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid LC-ESI-QQ , negative-QTOFsplash10-0udi-1900000000-b34932b6f2ee5bca96972017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid LC-ESI-QQ , negative-QTOFsplash10-0a4i-9100000000-cdbb3586477ed705b4d72017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid LC-ESI-QQ , negative-QTOFsplash10-0a4i-9000000000-022ab74e163dd752d0542017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid LC-ESI-QQ , negative-QTOFsplash10-0006-9000000000-187f6a4024ca6e9011882017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid LC-ESI-QQ , negative-QTOFsplash10-0006-9000000000-e8d8003bcda8aa818d4e2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid , negative-QTOFsplash10-0a4i-9100000000-79f60e90c0ce32d976b32017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid 35V, Negative-QTOFsplash10-0a4i-9200000000-b3917779cc00018df7d02021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid 10V, Positive-QTOFsplash10-002e-9000000000-d601a5284802b5564ac82021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid 40V, Positive-QTOFsplash10-0udi-9000000000-128801e4b0f2e4d5344b2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid 35V, Negative-QTOFsplash10-0a4i-9100000000-81d99c8d5304bd26a0cd2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 3-Hydroxybutyric acid 35V, Negative-QTOFsplash10-0a4i-9200000000-fc7c6bb126f183e27ff02021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 3-Hydroxybutyric acid 10V, Positive-QTOFsplash10-00kr-9100000000-889e2968ad4fd52cdd922017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 3-Hydroxybutyric acid 20V, Positive-QTOFsplash10-05n0-9000000000-6c660170b8f4aa6bcd022017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 3-Hydroxybutyric acid 40V, Positive-QTOFsplash10-0006-9000000000-c39869c905b7e93b7f972017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 3-Hydroxybutyric acid 10V, Negative-QTOFsplash10-0zfr-9800000000-532ea53160d6ca2efcaa2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 3-Hydroxybutyric acid 20V, Negative-QTOFsplash10-0pbi-9200000000-b2c59fd56b1a1d713ea92017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 3-Hydroxybutyric acid 40V, Negative-QTOFsplash10-0a4l-9000000000-29e8d104108ac71cd6402017-09-01Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 600 MHz, H2O, experimental)2012-12-04Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-04Wishart 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
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified60.0 +/- 20.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified40.0 +/- 10.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified323.0 +/- 29.0 uMAdult (>18 years old)FemaleNormal details
BloodDetected and Quantified36 +/- 20 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified86 +/- 53 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified38 +/- 31 uMAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified<500 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified260-5040 uMInfant (0-1 year old)Not Specified
Normal
details
BloodDetected and Quantified50.0 (0.0-100.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified46.5 +/- 10.1 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified<600 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified<150 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified100.0 +/- 90.0 uMAdult (>18 years old)FemaleNormal details
BloodDetected and Quantified565.0 +/- 255.0 uMNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified36.0 (13.0-95.0) uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified80-3600 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified64.88(60.4) uMAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified200 +/- 120 uMChildren (1-13 years old)Both
Normal
details
BloodDetected and Quantified170 +/- 110 uMChildren (1-13 years old)Both
Normal
details
BloodDetected and Quantified10.60-143.2 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified500-3000 uMChildren (1-13 years old)Male
Normal
details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified76.9 +/- 66.3 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified180.0 (22.0-700.0) uMAdult (>18 years old)BothNormal details
Cellular CytoplasmDetected and Quantified1300 (1200-1400) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified34 +/- 31 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified50.0 (4.0 - 96.0) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified286 (207-365) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified46.4 +/- 23.9 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified48.0 +/- 48.0 uMAdult (>18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified22.0932 +/- 9.606 nmol/g wet fecesNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
SalivaDetected and Quantified5.00 +/- 4.25 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not SpecifiedNormal details
SalivaDetected and Quantified2.64 +/- 1.83 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified3.22 +/- 2.77 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified3.88 +/- 4.26 uMAdult (>18 years old)BothNormal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified16.44 +/- 19.52 uMAdult (>18 years old)FemaleNormal 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
SalivaDetected and Quantified4.71 +/- 7.21 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
UrineDetected and Quantified0.5-9.8 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified<99 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified1.4-2.7 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified<54.301 umol/mmol creatinineInfant (0-1 year old)MaleNormal details
UrineDetected and Quantified180.280 +/- 115.119 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
details
UrineDetected and Quantified27.151 +/- 14.118 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
details
UrineDetected and Quantified272.592 +/- 419.205 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
details
UrineDetected and Quantified3.4 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified3397.0835 +/- 5584.336 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
details
UrineDetected and Quantified616.862 +/- 982.852 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
details
UrineDetected and Quantified7-120 umol/mmol creatinineChildren (1 - 13 years old)BothNormal details
UrineDetected and Quantified7569.588 +/- 3599.0840 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
details
UrineDetected and Quantified0.72-23.3 umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified4.02(0.78-12.39) umol/mmol creatinineNewborn (0-30 days old)FemaleNormal details
UrineDetected and Quantified3.14(0.71-23.84) umol/mmol creatinineNewborn (0-30 days old)MaleNormal details
UrineDetected and Quantified2.60 +/- 3.50 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified27.151 +/- 14.118 umol/mmol creatinineChildren (1-13 years old)Not Specified
Normal
details
UrineDetected and Quantified272.592 +/- 419.205 umol/mmol creatinineChildren (1-13 years old)Not Specified
Normal
details
UrineDetected and Quantified636.410 +/- 982.852 umol/mmol creatinineChildren (1-13 years old)Not Specified
Normal
details
UrineDetected and Quantified6.651 +/- 7.809 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
details
UrineDetected and Quantified180.4 +/- 115.00 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified3397.0835 +/- 5584.336 umol/mmol creatinineChildren (1-13 years old)Not Specified
Normal
details
UrineDetected and Quantified7569.588 +/- 3599.0840 umol/mmol creatinineChildren (1-13 years old)Not Specified
Normal
details
UrineDetected and Quantified13.0 (1.0-27.0) umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified1.4 +/- 1.3 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.7 (0-2.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<21.17 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified38.9 +/- 13.9 umol/mmol creatinineAdult (>18 years old)Male
Normal
details
UrineDetected and Quantified32.3 +/- 8.7 umol/mmol creatinineAdult (>18 years old)Female
Normal
details
UrineDetected and Quantified48.0 (0.00-200.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.4-2.2 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified1.62 (0.90-5.40) umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • Analysis of 40 NI...
details
UrineDetected and Quantified3.6 (1.3-6.4) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified2.257 +/- 1.254 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified2.0 (0.5-9.8) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified1.4 (0.1-2.7) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified1.3 (0.1-5.8) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified29.712 +/- 14.532 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
details
UrineDetected and Quantified0.62 (0.0-12.09) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified2.24 (0.0-17.6) umol/mmol creatinineAdult (>18 years old)Female
Normal
details
UrineDetected and Quantified3.6 (1.3-6.4) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified2.0 (0.6-5.6) umol/mmol creatinineInfant (0-1 year old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified235.0 +/- 17.0 uMAdult (>18 years old)FemaleObesity details
BloodDetected and Quantified35.1 +/- 33.9 uMAdult (>18 years old)BothHeart Transplant details
BloodDetected and Quantified62.7 (85.0) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
BloodDetected and Quantified21.0 (19.4) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified500 uMAdult (>18 years old)Male
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified660 uMAdolescent (13-18 years old)Female
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified200-2800 uMInfant (0-1 year old)Female
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected and Quantified770-2900 uMChildren (1-13 years old)Both
3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Oesophageal cancer
details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia
details
BloodDetected and Quantified220 uMInfant (0-1 year old)Not Specified
Very long-chain acyl-CoA dehydrogenase deficiency (vLCAD)
details
BloodDetected and Quantified80-200 uMInfant (0-1 year old)Not Specified
Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency
details
BloodDetected and Quantified700-1340 uMInfant (0-1 year old)Not Specified
Medium chain acyl-CoA dehydrogenase deficiency (MCAD)
details
BloodDetected and Quantified1800 uMInfant (0-1 year old)MalePyruvate dehydrogenase phosphatase deficiency details
BloodDetected and Quantified<100 uMChildren (1-13 years old)MaleCarnitine palmitoyltransferase deficiency I details
BloodDetected and Quantified1500.0 +/- 100.0 uMChildren (1-13 years old)Both
Diabetes
details
BloodDetected and Quantified0-380 uMInfant (0-1 year old)Not Specified
Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency
details
BloodDetected and Quantified1490-5040 uMInfant (0-1 year old)Not Specified
Ketotic hypoglycemia
details
BloodDetected and Quantified10-1380 uMInfant (0-1 year old)Female3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD) details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
BloodDetected and Quantified35.5 +/- 92.2 uMAdult (>18 years old)Both
Diabetes
details
BloodDetected and Quantified17.6 +/- 3.84 uMAdult (>18 years old)Both
Schizophrenia
details
BloodDetected and Quantified7700.0 +/- 300.0 uMChildren (1-13 years old)Both
Diabetes
details
BloodDetected and Quantified54.57 +/- 64.53 uMAdult (>18 years old)Female
Pregnancy with fetuses with trisomy 18
details
BloodDetected and Quantified32.9 +/- 53.42 uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified0-200 uMInfant (0-1 year old)Male3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD) details
BloodDetected and Quantified31.6 (19.1) uMAdult (>18 years old)FemaleEarly preeclampsia details
BloodDetected and Quantified27.0 (16.2) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified64 uMInfant (0-1 year old)Female
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
details
BloodDetected and Quantified100 uMNewborn (0-30 days old)Male2-Ketoglutarate dehydrogenase complex deficiency details
BloodDetected and Quantified120-2000 uMChildren (1-13 years old)Male2-Ketoglutarate dehydrogenase complex deficiency details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Pancreatic cancer
details
BloodDetected and Quantified33.63 (42.57) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified30.15 (37.69) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified29.61(44.27) uMAdult (>18 years old)BothHeart failure with reduced ejection fraction details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia
details
BloodDetected and Quantified49.9 (46.7) uMAdult (>18 years old)FemaleLate-onset preeclampsia details
BloodDetected and Quantified29.7 (19.1) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified130 +/- 80 uMChildren (1-13 years old)Both
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
details
BloodDetected and Quantified300 +/- 390 uMChildren (1-13 years old)Both
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
details
BloodDetected and Quantified300 +/- 200 uMChildren (1-13 years old)Both
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
details
BloodDetected and Quantified250 +/- 160 uMChildren (1-13 years old)Both
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
By NMR. Significantly decreased vs control.
details
BloodDetected and Quantified1400.0 +/- 100.0 uMAdult (>18 years old)Both
Diabetes
details
BloodDetected and Quantified7700.0 +/- 200.0 uMAdult (>18 years old)Both
Diabetes
details
BloodDetected and Quantified5700 (4300-6000) uMAdult (>18 years old)BothDiabetic ketoacidosis details
BloodDetected and Quantified20-50 uMChildren (1-13 years old)Male
3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency
details
Cellular CytoplasmDetected and Quantified2600 (2500-2700) uMAdult (>18 years old)BothAnoxia details
Cerebrospinal Fluid (CSF)Detected and Quantified430 (359-501) uMAdult (>18 years old)Both
Meningitis
details
Cerebrospinal Fluid (CSF)Detected and Quantified6360 (5350-7370) uMAdult (>18 years old)Both
Diabetes
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Ulcerative colitis
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Crohn disease
details
SalivaDetected and Quantified5.04 +/- 3.02 uMAdult (>18 years old)BothTooth alignment disorders
    • Sugimoto et al. (...
details
SalivaDetected and Quantified6.62 +/- 0.72 uMAdult (>18 years old)MaleAlzheimer's disease details
SalivaDetected and Quantified6.37 +/- 1.75 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
SalivaDetected and Quantified24.25 +/- 32.08 uMAdult (>18 years old)BothLewy body disease details
UrineDetected and Quantified11.946 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified16.29 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified27.151 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified31.495 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified56.473 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Medium Chain Acyl-CoA Dehydrogenase Deficiency
details
UrineDetected and Quantified351.872 umol/mmol creatinineInfant (0-1 year old)MaleFumarase deficiency details
UrineDetected and Quantified45000 umol/mmol creatinineChildren (1-13 years old)Not AvailablePyruvate carboxylase deficiency details
UrineDetected and Quantified8359 umol/mmol creatinineInfant (0-1 year old)Not AvailablePyruvate carboxylase deficiency details
UrineDetected and Quantified3-1050 umol/mmol creatinineChildren (1 - 13 years old)BothMedium Chain Acyl-CoA Dehydrogenase Deficiency details
UrineDetected and Quantified55.399-1634.282 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedMedium Chain Acyl-CoA Dehydrogenase Deficiency details
UrineDetected and Quantified170-390 umol/mmol creatinineChildren (1-13 years old)Male3-Hydroxyisobutyric acid dehydrogenase deficiency details
UrineDetected and Quantified35.352 +/- 40.756 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
UrineDetected and Quantified51.0431-56.473 umol/mmol creatinineInfant (0-1 year old)Not Specified3-Hydroxydicarboxylic aciduria details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
UrineDetected and Quantified224 umol/mmol creatinineAdult (>18 years old)BothDiabetes details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia
details
UrineDetected and Quantified30.883 +/- 22.266 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
UrineDetected and Quantified74.311 +/- 155.898 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Analysis of 30 no...
details
Associated Disorders and Diseases
Disease References
Diabetes mellitus type 2
  1. Sheikh-Ali M, Karon BS, Basu A, Kudva YC, Muller LA, Xu J, Schwenk WF, Miles JM: Can serum beta-hydroxybutyrate be used to diagnose diabetic ketoacidosis? Diabetes Care. 2008 Apr;31(4):643-7. doi: 10.2337/dc07-1683. Epub 2008 Jan 9. [PubMed:18184896 ]
  2. Tasker RC, Lutman D, Peters MJ: Hyperventilation in severe diabetic ketoacidosis. Pediatr Crit Care Med. 2005 Jul;6(4):405-11. [PubMed:15982426 ]
  3. Bales JR, Higham DP, Howe I, Nicholson JK, Sadler PJ: Use of high-resolution proton nuclear magnetic resonance spectroscopy for rapid multi-component analysis of urine. Clin Chem. 1984 Mar;30(3):426-32. [PubMed:6321058 ]
  4. del Carmen Crespillo M, Olveira G, de Adana MS, Rojo-Martinez G, Garcia-Aleman J, Olvera P, Soriguer F, Munoz A: Metabolic effects of an enteral nutrition formula for diabetes: comparison with standard formulas in patients with type 1 diabetes. Clin Nutr. 2003 Oct;22(5):483-7. [PubMed:14512036 ]
Obesity
  1. Vice E, Privette JD, Hickner RC, Barakat HA: Ketone body metabolism in lean and obese women. Metabolism. 2005 Nov;54(11):1542-5. [PubMed:16253646 ]
Pancreatic cancer
  1. OuYang D, Xu J, Huang H, Chen Z: Metabolomic profiling of serum from human pancreatic cancer patients using 1H NMR spectroscopy and principal component analysis. Appl Biochem Biotechnol. 2011 Sep;165(1):148-54. doi: 10.1007/s12010-011-9240-0. Epub 2011 Apr 20. [PubMed:21505807 ]
  2. Zhang L, Jin H, Guo X, Yang Z, Zhao L, Tang S, Mo P, Wu K, Nie Y, Pan Y, Fan D: Distinguishing pancreatic cancer from chronic pancreatitis and healthy individuals by (1)H nuclear magnetic resonance-based metabonomic profiles. Clin Biochem. 2012 Sep;45(13-14):1064-9. doi: 10.1016/j.clinbiochem.2012.05.012. Epub 2012 May 19. [PubMed:22613268 ]
Colorectal cancer
  1. Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ]
  2. 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 ]
Schizophrenia
  1. Fukushima T, Iizuka H, Yokota A, Suzuki T, Ohno C, Kono Y, Nishikiori M, Seki A, Ichiba H, Watanabe Y, Hongo S, Utsunomiya M, Nakatani M, Sadamoto K, Yoshio T: Quantitative analyses of schizophrenia-associated metabolites in serum: serum D-lactate levels are negatively correlated with gamma-glutamylcysteine in medicated schizophrenia patients. PLoS One. 2014 Jul 8;9(7):e101652. doi: 10.1371/journal.pone.0101652. eCollection 2014. [PubMed:25004141 ]
  2. Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]
  3. Cai HL, Li HD, Yan XZ, Sun B, Zhang Q, Yan M, Zhang WY, Jiang P, Zhu RH, Liu YP, Fang PF, Xu P, Yuan HY, Zhang XH, Hu L, Yang W, Ye HS: Metabolomic analysis of biochemical changes in the plasma and urine of first-episode neuroleptic-naive schizophrenia patients after treatment with risperidone. J Proteome Res. 2012 Aug 3;11(8):4338-50. doi: 10.1021/pr300459d. Epub 2012 Jul 26. [PubMed:22800120 ]
Early preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
Pregnancy
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
  2. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
  3. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester Down syndrome prediction. Am J Obstet Gynecol. 2013 May;208(5):371.e1-8. doi: 10.1016/j.ajog.2012.12.035. Epub 2013 Jan 8. [PubMed:23313728 ]
  4. Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240 ]
  5. Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
Late-onset preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
2-Ketoglutarate dehydrogenase complex deficiency
  1. Guffon N, Lopez-Mediavilla C, Dumoulin R, Mousson B, Godinot C, Carrier H, Collombet JM, Divry P, Mathieu M, Guibaud P: 2-Ketoglutarate dehydrogenase deficiency, a rare cause of primary hyperlactataemia: report of a new case. J Inherit Metab Dis. 1993;16(5):821-30. [PubMed:8295396 ]
3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency
  1. Morris AA, Lascelles CV, Olpin SE, Lake BD, Leonard JV, Quant PA: Hepatic mitochondrial 3-hydroxy-3-methylglutaryl-coenzyme a synthase deficiency. Pediatr Res. 1998 Sep;44(3):392-6. doi: 10.1203/00006450-199809000-00021. [PubMed:9727719 ]
3-Hydroxyacyl-CoA dehydrogenase deficiency
  1. Hsu BY, Kelly A, Thornton PS, Greenberg CR, Dilling LA, Stanley CA: Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndrome. J Pediatr. 2001 Mar;138(3):383-9. doi: 10.1067/mpd.2001.111818. [PubMed:11241047 ]
  2. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
  3. Popa FI, Perlini S, Teofoli F, Degani D, Funghini S, La Marca G, Rinaldo P, Vincenzi M, Antoniazzi F, Boner A, Camilot M: 3-hydroxyacyl-coenzyme a dehydrogenase deficiency: identification of a new mutation causing hyperinsulinemic hypoketotic hypoglycemia, altered organic acids and acylcarnitines concentrations. JIMD Rep. 2012;2:71-7. doi: 10.1007/8904_2011_50. Epub 2011 Sep 6. [PubMed:23430856 ]
Long-chain-3-hydroxyacyl CoA dehydrogenase deficiency
  1. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency
  1. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
Ketotic hypoglycemia
  1. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
  1. Zschocke J, Penzien JM, Bielen R, Casals N, Aledo R, Pie J, Hoffmann GF, Hegardt FG, Mayatepek E: The diagnosis of mitochondrial HMG-CoA synthase deficiency. J Pediatr. 2002 Jun;140(6):778-80. doi: 10.1067/mpd.2002.123854. [PubMed:12072887 ]
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
  1. Benkert AR, Young M, Robinson D, Hendrickson C, Lee PA, Strauss KA: Severe Salt-Losing 3beta-Hydroxysteroid Dehydrogenase Deficiency: Treatment and Outcomes of HSD3B2 c.35G>A Homozygotes. J Clin Endocrinol Metab. 2015 Aug;100(8):E1105-15. doi: 10.1210/jc.2015-2098. Epub 2015 Jun 16. [PubMed:26079780 ]
Pyruvate dehydrogenase phosphatase deficiency
  1. Robinson BH, Sherwood WG: Pyruvate dehydrogenase phosphatase deficiency: a cause of congenital chronic lactic acidosis in infancy. Pediatr Res. 1975 Dec;9(12):935-9. doi: 10.1203/00006450-197512000-00015. [PubMed:172850 ]
Carnitine palmitoyltransferase I deficiency
  1. Olpin SE, Allen J, Bonham JR, Clark S, Clayton PT, Calvin J, Downing M, Ives K, Jones S, Manning NJ, Pollitt RJ, Standing SJ, Tanner MS: Features of carnitine palmitoyltransferase type I deficiency. J Inherit Metab Dis. 2001 Feb;24(1):35-42. [PubMed:11286380 ]
Anoxia
  1. Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed:8579834 ]
Meningitis
  1. Subramanian A, Gupta A, Saxena S, Gupta A, Kumar R, Nigam A, Kumar R, Mandal SK, Roy R: Proton MR CSF analysis and a new software as predictors for the differentiation of meningitis in children. NMR Biomed. 2005 Jun;18(4):213-25. [PubMed:15627241 ]
Ulcerative colitis
  1. Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
Crohn's disease
  1. Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
Alzheimer's disease
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Frontotemporal dementia
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Lewy body disease
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Medium Chain Acyl-CoA Dehydrogenase Deficiency
  1. Tserng KY, Jin SJ, Kerr DS, Hoppel CL: Abnormal urinary excretion of unsaturated dicarboxylic acids in patients with medium-chain acyl-CoA dehydrogenase deficiency. J Lipid Res. 1990 May;31(5):763-71. [PubMed:2380628 ]
  2. Gregersen N, Kolvraa S, Rasmussen K, Mortensen PB, Divry P, David M, Hobolth N: General (medium-chain) acyl-CoA dehydrogenase deficiency (non-ketotic dicarboxylic aciduria): quantitative urinary excretion pattern of 23 biologically significant organic acids in three cases. Clin Chim Acta. 1983 Aug 15;132(2):181-91. [PubMed:6616873 ]
  3. Duran M, De Klerk JB, Wadman SK, Bruinvis L, Ketting D: The differential diagnosis of dicarboxylic aciduria. J Inherit Metab Dis. 1984;7 Suppl 1:48-51. [PubMed:6434845 ]
  4. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
Fumarase deficiency
  1. Bastug O, Kardas F, Ozturk MA, Halis H, Memur S, Korkmaz L, Tag Z, Gunes T: A rare cause of opistotonus; fumaric aciduria: The first case presentation in Turkey. Turk Pediatri Ars. 2014 Mar 1;49(1):74-6. doi: 10.5152/tpa.2014.442. eCollection 2014 Mar. [PubMed:26078636 ]
3-Hydroxyisobutyric acid dehydrogenase deficiency
  1. Ko FJ, Nyhan WL, Wolff J, Barshop B, Sweetman L: 3-Hydroxyisobutyric aciduria: an inborn error of valine metabolism. Pediatr Res. 1991 Oct;30(4):322-6. doi: 10.1203/00006450-199110000-00006. [PubMed:1956714 ]
3-Hydroxydicarboxylic aciduria
  1. Tserng KY, Jin SJ, Kerr DS, Hoppel CL: Urinary 3-hydroxydicarboxylic acids in pathophysiology of metabolic disorders with dicarboxylic aciduria. Metabolism. 1991 Jul;40(7):676-82. [PubMed:1870421 ]
Pyruvate carboxylase deficiency
  1. Habarou F, Brassier A, Rio M, Chretien D, Monnot S, Barbier V, Barouki R, Bonnefont JP, Boddaert N, Chadefaux-Vekemans B, Le Moyec L, Bastin J, Ottolenghi C, de Lonlay P: Pyruvate carboxylase deficiency: An underestimated cause of lactic acidosis. Mol Genet Metab Rep. 2014 Nov 28;2:25-31. doi: 10.1016/j.ymgmr.2014.11.001. eCollection 2015 Mar. [PubMed:28649521 ]
Associated OMIM IDs
  • 125853 (Diabetes mellitus type 2)
  • 601665 (Obesity)
  • 260350 (Pancreatic cancer)
  • 114500 (Colorectal cancer)
  • 181500 (Schizophrenia)
  • 203740 (2-Ketoglutarate dehydrogenase complex deficiency)
  • 605911 (3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency)
  • 231530 (3-Hydroxyacyl-CoA dehydrogenase deficiency)
  • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
  • 201810 (Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency)
  • 608782 (Pyruvate dehydrogenase phosphatase deficiency)
  • 255120 (Carnitine palmitoyltransferase I deficiency)
  • 266600 (Crohn's disease)
  • 104300 (Alzheimer's disease)
  • 600274 (Frontotemporal dementia)
  • 610247 (Eosinophilic esophagitis)
  • 201450 (Medium Chain Acyl-CoA Dehydrogenase Deficiency)
  • 606812 (Fumarase deficiency)
  • 266150 (Pyruvate carboxylase deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB021869
KNApSAcK IDNot Available
Chemspider ID83181
KEGG Compound IDC01089
BioCyc IDCPD-335
BiGG ID36784
Wikipedia LinkBeta-Hydroxybutyric_acid
METLIN IDNot Available
PubChem Compound92135
PDB IDNot Available
ChEBI ID17066
Food Biomarker OntologyNot Available
VMH IDBHB
MarkerDB IDMDB00000005
Good Scents IDNot Available
References
Synthesis ReferenceLe Sann, Christine; Munoz, Dulce M.; Saunders, Natalie; Simpson, Thomas J.; Smith, David I.; Soulas, Florilene; Watts, Paul; Willis, Christine L. Assembly intermediates in polyketide biosynthesis: enantioselective syntheses of b-hydroxycarbonyl compounds. Org Biomol Chem. 2005 May 7;3(9):1719-28. Epub 2005 Mar 31. Pubmed: 15858656
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Pan JW, Rothman TL, Behar KL, Stein DT, Hetherington HP: Human brain beta-hydroxybutyrate and lactate increase in fasting-induced ketosis. J Cereb Blood Flow Metab. 2000 Oct;20(10):1502-7. [PubMed:11043913 ]
  2. Pan JW, Telang FW, Lee JH, de Graaf RA, Rothman DL, Stein DT, Hetherington HP: Measurement of beta-hydroxybutyrate in acute hyperketonemia in human brain. J Neurochem. 2001 Nov;79(3):539-44. [PubMed:11701757 ]
  3. Plecko B, Stoeckler-Ipsiroglu S, Schober E, Harrer G, Mlynarik V, Gruber S, Moser E, Moeslinger D, Silgoner H, Ipsiroglu O: Oral beta-hydroxybutyrate supplementation in two patients with hyperinsulinemic hypoglycemia: monitoring of beta-hydroxybutyrate levels in blood and cerebrospinal fluid, and in the brain by in vivo magnetic resonance spectroscopy. Pediatr Res. 2002 Aug;52(2):301-6. [PubMed:12149510 ]
  4. Raunio RP, Leivo PV, Kuusinen AM: Bioluminescent assay of D-3-hydroxybutyrate in serum. J Biolumin Chemilumin. 1986 Jun;1(1):11-4. [PubMed:3503522 ]
  5. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed:2026685 ]
  6. Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed:8412012 ]
  7. Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed:8579834 ]
  8. Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. [PubMed:9693263 ]
  9. Frenkel G, Peterson RN, Freund M: Oxidative and glycolytic metabolism of semen components by washed guinea pig spermatozoa. Fertil Steril. 1975 Feb;26(2):144-7. [PubMed:1126459 ]
  10. Abrahamsson K, Eriksson BO, Holme E, Jodal U, Jonsson A, Lindstedt S: Pivalic acid-induced carnitine deficiency and physical exercise in humans. Metabolism. 1996 Dec;45(12):1501-7. [PubMed:8969283 ]
  11. Teresinski G, Buszewicz G, Madro R: The influence of ethanol on the level of ketone bodies in hypothermia. Forensic Sci Int. 2002 Jun 25;127(1-2):88-96. [PubMed:12098531 ]
  12. Nalecz KA, Miecz D, Berezowski V, Cecchelli R: Carnitine: transport and physiological functions in the brain. Mol Aspects Med. 2004 Oct-Dec;25(5-6):551-67. [PubMed:15363641 ]
  13. Blomqvist G, Alvarsson M, Grill V, Von Heijne G, Ingvar M, Thorell JO, Stone-Elander S, Widen L, Ekberg K: Effect of acute hyperketonemia on the cerebral uptake of ketone bodies in nondiabetic subjects and IDDM patients. Am J Physiol Endocrinol Metab. 2002 Jul;283(1):E20-8. [PubMed:12067838 ]
  14. Krotkiewski M: Value of VLCD supplementation with medium chain triglycerides. Int J Obes Relat Metab Disord. 2001 Sep;25(9):1393-400. [PubMed:11571605 ]
  15. Kamijo T, Indo Y, Souri M, Aoyama T, Hara T, Yamamoto S, Ushikubo S, Rinaldo P, Matsuda I, Komiyama A, Hashimoto T: Medium chain 3-ketoacyl-coenzyme A thiolase deficiency: a new disorder of mitochondrial fatty acid beta-oxidation. Pediatr Res. 1997 Nov;42(5):569-76. [PubMed:9357925 ]
  16. Geary N, Grotschel H, Scharrer E: Blood metabolites and feeding during postinsulin hypophagia. Am J Physiol. 1982 Sep;243(3):R304-11. [PubMed:7051864 ]
  17. Yeh YY, Sheehan PM: Preferential utilization of ketone bodies in the brain and lung of newborn rats. Fed Proc. 1985 Apr;44(7):2352-8. [PubMed:3884391 ]
  18. Kita K, Ishimaru K, Teraoka M, Yanase H, Kato N: Properties of poly(3-hydroxybutyrate) depolymerase from a marine bacterium, Alcaligenes faecalis AE122. Appl Environ Microbiol. 1995 May;61(5):1727-30. [PubMed:7646009 ]
  19. Kim BS, Lee SC, Lee SY, Chang HN, Chang YK, Woo SI: Production of poly(3-hydroxybutyric acid) by fed-batch culture of Alcaligenes eutrophus with glucose concentration control. Biotechnol Bioeng. 1994 Apr 15;43(9):892-8. doi: 10.1002/bit.260430908. [PubMed:18615882 ]
  20. 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 ]
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Enzymes

General function:
Involved in sulfotransferase activity
Specific function:
Sulfotransferase that utilizes 3'-phospho-5'-adenylyl sulfate (PAPS) as sulfonate donor to catalyze the sulfate conjugation of many hormones, neurotransmitters, drugs and xenobiotic compounds. Sulfonation increases the water solubility of most compounds, and therefore their renal excretion, but it can also result in bioactivation to form active metabolites. Sulfates hydroxysteroids like DHEA. Isoform 1 preferentially sulfonates cholesterol, and isoform 2 avidly sulfonates pregnenolone but not cholesterol.
Gene Name:
SULT2B1
Uniprot ID:
O00204
Molecular weight:
39598.595
Reactions
3-Hydroxybutyric acid → 3-(sulfooxy)butanoic aciddetails
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform glucuronidates bilirubin IX-alpha to form both the IX-alpha-C8 and IX-alpha-C12 monoconjugates and diconjugate. Is also able to catalyze the glucuronidation of 17beta-estradiol, 17alpha-ethinylestradiol, 1-hydroxypyrene, 4-methylumbelliferone, 1-naphthol, paranitrophenol, scopoletin, and umbelliferone.
Gene Name:
UGT1A1
Uniprot ID:
P22309
Molecular weight:
59590.91
Reactions
3-Hydroxybutyric acid → 6-[(1-carboxypropan-2-yl)oxy]-3,4,5-trihydroxyoxane-2-carboxylic aciddetails
3-Hydroxybutyric acid → 3,4,5-trihydroxy-6-[(3-hydroxybutanoyl)oxy]oxane-2-carboxylic aciddetails
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
BDH1
Uniprot ID:
Q02338
Molecular weight:
38156.77
Reactions
3-Hydroxybutyric acid + NAD → Acetoacetic acid + NADHdetails
3-Hydroxybutyric acid + NAD → Acetoacetic acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Dehydrogenase that mediates the formation of 2,5-dihydroxybenzoic acid (2,5-DHBA), a siderophore that shares structural similarities with bacterial enterobactin and associates with LCN2, thereby playing a key role in iron homeostasis and transport. Also acts as a 3-hydroxybutyrate dehydrogenase (By similarity).
Gene Name:
BDH2
Uniprot ID:
Q9BUT1
Molecular weight:
26723.57
Reactions
3-Hydroxybutyric acid + NAD → Acetoacetic acid + NADHdetails
3-Hydroxybutyric acid + NAD → Acetoacetic acid + NADH + Hydrogen Iondetails
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
DKFZp434G1411
Uniprot ID:
Q9NT06
Molecular weight:
16899.1
References
  1. Gibson KM, Lee CF, Kamali V, Johnston K, Beaudet AL, Craigen WJ, Powell BR, Schwartz R, Tsai MY, Tuchman M: 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency as detected by radiochemical assay in cell extracts by thin-layer chromatography, and identification of three new cases. Clin Chem. 1990 Feb;36(2):297-303. [PubMed:2302772 ]