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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:57 UTC
HMDB IDHMDB0000008
Secondary Accession Numbers
  • HMDB00008
Metabolite Identification
Common Name2-Hydroxybutyric acid
Description2-Hydroxybutyric acid (CAS: 600-15-7), also known as alpha-hydroxybutyrate, is an organic acid derived from alpha-ketobutyrate. alpha-Ketobutyrate is produced by amino acid catabolism (threonine and methionine) and glutathione anabolism (cysteine formation pathway) and is metabolized into propionyl-CoA and carbon dioxide (PMID: 20526369 ). 2-Hydroxybutyric acid is formed as a byproduct from the formation of alpha-ketobutyrate via a reaction catalyzed by lactate dehydrogenase (LDH) or alpha-hydroxybutyrate dehydrogenase (alphaHBDH). alpha-Hydroxybutyric acid is primarily produced in mammalian hepatic tissues that catabolize L-threonine or synthesize glutathione. Oxidative stress or detoxification of xenobiotics in the liver can dramatically increase the rate of hepatic glutathione synthesis. Under such metabolic stress conditions, supplies of L-cysteine for glutathione synthesis become limiting, so homocysteine is diverted from the transmethylation pathway (which forms methionine) into the transsulfuration pathway (which forms cystathionine). alpha-Ketobutyrate is released as a byproduct when cystathionine is cleaved into cysteine that is incorporated into glutathione. Chronic shifts in the rate of glutathione synthesis may be reflected by urinary excretion of 2-hydroxybutyrate. 2-Hydroxybutyrate is an early marker for both insulin resistance and impaired glucose regulation that appears to arise due to increased lipid oxidation and oxidative stress (PMID: 20526369 ). 2-Hydroxybutyric acid is often found in the urine of patients suffering from lactic acidosis and ketoacidosis. 2-Hydroxybutyric acid generally appears at high concentrations in situations related to deficient energy metabolism (e.g. birth asphyxia) and also in inherited metabolic diseases affecting the central nervous system during neonatal development, such as "cerebral" lactic acidosis, glutaric aciduria type II, dihydrolipoyl dehydrogenase (E3) deficiency, and propionic acidemia. More recently it has been noted that elevated levels of alpha-hydroxybutyrate in the plasma is a good marker for early-stage type II diabetes (PMID: 19166731 ). It was concluded from studies done in the mid-1970s that an increased NADH2/NAD ratio was the most important factor for the production of 2-hydroxybutyric acid (PMID: 168632 ).
Structure
Data?1676999667
Synonyms
ValueSource
(S)-2-Hydroxybutanoic acidChEBI
2-HydroxybutyrateChEBI
2-Hydroxybutyric acidChEBI
L-2-Hydroxybutanoic acidChEBI
L-2-Hydroxybutyric acidChEBI
L-alpha-Hydroxybutanoic acidChEBI
L-alpha-Hydroxybutyric acidChEBI
(S)-2-HydroxybutanoateGenerator
L-2-HydroxybutanoateGenerator
L-2-HydroxybutyrateGenerator
L-a-HydroxybutanoateGenerator
L-a-Hydroxybutanoic acidGenerator
L-alpha-HydroxybutanoateGenerator
L-Α-hydroxybutanoateGenerator
L-Α-hydroxybutanoic acidGenerator
L-a-HydroxybutyrateGenerator
L-a-Hydroxybutyric acidGenerator
L-alpha-HydroxybutyrateGenerator
L-Α-hydroxybutyrateGenerator
L-Α-hydroxybutyric acidGenerator
(S)-2-HydroxybutyrateGenerator
2-HydroxybutanoateGenerator
(+)-2-Hydroxy-n-butyric acidHMDB
(+)-2-Hydroxybutanoic acidHMDB
(+)-2-Hydroxybutyric acidHMDB
(+)-alpha-Hydroxybutyric acidHMDB
(+)-α-Hydroxybutyric acidHMDB
(2S)-2-Hydroxybutanoic acidHMDB
(S)-(+)-2-Hydroxybutanoic acidHMDB
(S)-2-Hydroxybutyric acidHMDB
2-Hydroxybutanoic acidHMDB
alpha-Hydroxy-n-butyric acidHMDB
alpha-Hydroxybutanoic acidHMDB
alpha-Hydroxybutyric acidHMDB
α-Hydroxy-n-butyric acidHMDB
α-Hydroxybutanoic acidHMDB
α-Hydroxybutyric acidHMDB
Chemical FormulaC4H8O3
Average Molecular Weight104.105
Monoisotopic Molecular Weight104.047344118
IUPAC Name(2S)-2-hydroxybutanoic acid
Traditional Name(S)-2-hydroxybutyric acid
CAS Registry Number3347-90-8
SMILES
[H]OC(=O)[C@@]([H])(O[H])C([H])([H])C([H])([H])[H]
InChI Identifier
InChI=1S/C4H8O3/c1-2-3(5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1
InChI KeyAFENDNXGAFYKQO-VKHMYHEASA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as alpha hydroxy acids and derivatives. These are organic compounds containing a carboxylic acid substituted with a hydroxyl group on the adjacent carbon.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassHydroxy acids and derivatives
Sub ClassAlpha hydroxy acids and derivatives
Direct ParentAlpha hydroxy acids and derivatives
Alternative Parents
Substituents
  • Fatty acid
  • Alpha-hydroxy 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 Point44.2 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Baker124.66730932474
[M-H]-Not Available124.667http://allccs.zhulab.cn/database/detail?ID=AllCCS00001760
Predicted Molecular Properties
PropertyValueSource
Water Solubility484 g/LALOGPS
logP-0.16ALOGPS
logP0.051ChemAxon
logS0.67ALOGPS
pKa (Strongest Acidic)3.99ChemAxon
pKa (Strongest Basic)-3.8ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area57.53 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity23.36 m³·mol⁻¹ChemAxon
Polarizability9.98 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
DeepCCS[M+H]+120.60530932474
DeepCCS[M-H]-117.80430932474
DeepCCS[M-2H]-154.26130932474
DeepCCS[M+Na]+128.94530932474
AllCCS[M+H]+127.732859911
AllCCS[M+H-H2O]+123.432859911
AllCCS[M+NH4]+131.832859911
AllCCS[M+Na]+132.932859911
AllCCS[M-H]-123.632859911
AllCCS[M+Na-2H]-127.432859911
AllCCS[M+HCOO]-131.732859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
2-Hydroxybutyric acidCC[C@H](O)C(O)=O1796.3Standard polar33892256
2-Hydroxybutyric acidCC[C@H](O)C(O)=O928.5Standard non polar33892256
2-Hydroxybutyric acidCC[C@H](O)C(O)=O990.5Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
2-Hydroxybutyric acid,1TMS,isomer #1CC[C@H](O[Si](C)(C)C)C(=O)O1087.2Semi standard non polar33892256
2-Hydroxybutyric acid,1TMS,isomer #2CC[C@H](O)C(=O)O[Si](C)(C)C983.5Semi standard non polar33892256
2-Hydroxybutyric acid,2TMS,isomer #1CC[C@H](O[Si](C)(C)C)C(=O)O[Si](C)(C)C1135.8Semi standard non polar33892256
2-Hydroxybutyric acid,1TBDMS,isomer #1CC[C@H](O[Si](C)(C)C(C)(C)C)C(=O)O1313.5Semi standard non polar33892256
2-Hydroxybutyric acid,1TBDMS,isomer #2CC[C@H](O)C(=O)O[Si](C)(C)C(C)(C)C1207.3Semi standard non polar33892256
2-Hydroxybutyric acid,2TBDMS,isomer #1CC[C@H](O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1575.5Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - 2-Hydroxybutyric acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-001j-0900000000-44dd1bf8072e5731bac42020-02-05HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Hydroxybutyric acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-00e9-9700000000-93eba027d5343e3d6ce12020-02-05HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (2 TMS)splash10-001i-1910000000-3bc9898b26df33cad2442020-02-05HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Hydroxybutyric acid EI-B (Non-derivatized)splash10-0a4i-9000000000-4d935da8e593ec61fd962020-02-05HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Hydroxybutyric acid GC-EI-TOF (Non-derivatized)splash10-001j-0900000000-44dd1bf8072e5731bac42020-02-05HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Hydroxybutyric acid GC-EI-TOF (Non-derivatized)splash10-00e9-9700000000-93eba027d5343e3d6ce12020-02-05HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (Non-derivatized)splash10-001i-1910000000-3bc9898b26df33cad2442020-02-05HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TMS_2_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TBDMS_2_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxybutyric acid Quattro_QQQ 10V, Negative-QTOF (Annotated)splash10-0zfr-9600000000-f688c3e6fdeb5f0270c52020-02-05HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxybutyric acid Quattro_QQQ 25V, Negative-QTOF (Annotated)splash10-0a4j-9200000000-e3b371c29b9ebed3199b2020-02-05HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxybutyric acid Quattro_QQQ 40V, Negative-QTOF (Annotated)splash10-0zfs-9700000000-bccfada2c97e04d344172020-02-05HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxybutyric acid EI-B (HITACHI RMU-7M) , Positive-QTOFsplash10-0a4i-9000000000-4d935da8e593ec61fd962020-02-05HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxybutyric acid LC-ESI-IT , negative-QTOFsplash10-0udi-2900000000-3c7915ea71886873f0242020-02-05HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxybutyric acid , negative-QTOFsplash10-0zfr-7900000000-d91c840306a06d4ea5132020-02-05HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxybutyric acid 40V, Negative-QTOFsplash10-0006-9000000000-94eb4d9d668d4d53231b2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxybutyric acid 10V, Negative-QTOFsplash10-0pb9-9500000000-a90d8674aafbec41b8b22021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxybutyric acid 20V, Negative-QTOFsplash10-0a4i-9400000000-7b0a49d8800ded4cdff42021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxybutyric acid 35V, Negative-QTOFsplash10-0zfr-7900000000-52227d0dd814179fde452021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxybutyric acid 35V, Negative-QTOFsplash10-0zmi-9600000000-8ea58ec01176ecce47352021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 10V, Positive-QTOFsplash10-0a4r-9400000000-7bdf5cb6c5aa8c1d607d2019-02-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 20V, Positive-QTOFsplash10-0a4r-9100000000-bf04b472392c16bd165c2019-02-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 40V, Positive-QTOFsplash10-052f-9000000000-483980f183cd4095b2f32019-02-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 10V, Negative-QTOFsplash10-0udi-5900000000-9185cef9251e02ac688c2019-02-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 20V, Negative-QTOFsplash10-0a4r-9200000000-ea836921fe44635e66c22019-02-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 40V, Negative-QTOFsplash10-0a4l-9000000000-cf8130e8094c63aa0e252019-02-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 10V, Positive-QTOFsplash10-052o-9000000000-d175dd460cd717e713502021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 20V, Positive-QTOFsplash10-0a4l-9000000000-0244ee6a1375f83df3a22021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 40V, Positive-QTOFsplash10-0006-9000000000-a4c46731c8286baabca92021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 10V, Negative-QTOFsplash10-0udi-2900000000-d938708d3f7f8d7127102021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 20V, Negative-QTOFsplash10-0ap0-9100000000-a0763e4b908fc98b58532021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 40V, Negative-QTOFsplash10-0a4i-9000000000-9077c11ebfee5a552d352021-09-24Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 600 MHz, H2O, experimental)2020-02-05Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-14Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2020-02-05Wishart 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
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Locations
  • Placenta
  • Prostate
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified8.00-80.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified26.9(32.92) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified8.00-80.0 uMAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Female
Normal
details
BloodDetected and Quantified54.0 (8.0-80.0) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified40.0 +/- 24.0 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified85.0 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified37 +/- 21 (10 - 76) uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified35.0 +/- 24.0 uMAdult (>18 years old)BothNormal 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
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 Quantified10.42 +/- 9.20 uMAdult (>18 years old)FemaleNormal details
SweatDetected but not QuantifiedNot QuantifiedAdult BothNormal details
UrineDetected and Quantified0.31-0.69 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified2.445 (0-4.89) umol/mmol creatinineNot SpecifiedBothNormal details
UrineDetected and Quantified0-3 umol/mmol creatinineInfant (0-1 year old)Male
Normal
details
UrineDetected and Quantified0-6.0 umol/mmol creatinineInfant (0-1 year old)Male
Normal
details
UrineDetected and Quantified0-6.1 umol/mmol creatinineInfant (0-1 year old)Female
Normal
details
UrineDetected and Quantified0-9.3 umol/mmol creatinineInfant (0-1 year old)Female
Normal
details
UrineDetected and Quantified3.29 +/- 3.529 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
details
UrineDetected and Quantified2.8 (1.2-6.9) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified5.0 (0.0-10.0) umol/mmol creatinineInfant (0-1 year old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified0.26-0.53 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified2.53 (1.56-6.08) umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • Analysis of 40 NI...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified26.96 +/- 13.11 uMAdult (>18 years old)Female
Pregnancy with fetuses with trisomy 18
details
BloodDetected and Quantified16.93 +/- 8.17 uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified21.4 (5.6) uMAdult (>18 years old)FemaleEarly preeclampsia details
BloodDetected and Quantified19.5 (7.4) uMAdult (>18 years old)FemalePregnancy details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
BloodDetected and Quantified17.13 (7.48) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified16.69 (6.25) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified28.0 (14.4) uMAdult (>18 years old)FemaleLate-onset preeclampsia details
BloodDetected and Quantified4.14(10.18) uMAdult (>18 years old)BothHeart failure with reduced ejection fraction details
BloodDetected and Quantified21.2 (7.5) uMAdult (>18 years old)FemalePregnancy details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia
details
BloodDetected and Quantified23.1 (14.1) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
BloodDetected and Quantified15.0 (7.4) uMAdult (>18 years old)FemalePregnancy details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
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 and Quantified7.26 +/- 4.47 uMAdult (>18 years old)MaleAlzheimer's disease details
SalivaDetected and Quantified7.30 +/- 1.32 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
SalivaDetected and Quantified8.12 +/- 5.32 uMAdult (>18 years old)BothLewy body disease details
UrineDetected and Quantified>25.0 umol/mmol creatinineAdult (>18 years old)BothPyruvate dehydrogenase deficiency
    • OMIM 238331
details
UrineDetected and Quantified8.699 +/- 10.366 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Schizophrenia
details
UrineDetected and Quantified248.732 umol/mmol creatinineInfant (0-1 year old)MaleDihydrolipoamide Dehydrogenase Deficiency details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal adenoma
details
Associated Disorders and Diseases
Disease References
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 ]
  3. 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 ]
  4. 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 ]
Schizophrenia
  1. 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 ]
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 ]
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 ]
Pyruvate dehydrogenase deficiency
  1. (). OMIM 238331. .
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Dihydrolipoamide Dehydrogenase Deficiency
  1. Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB021867
KNApSAcK IDNot Available
Chemspider ID389701
KEGG Compound IDC05984
BioCyc IDCPD-3564
BiGG IDNot Available
Wikipedia Link2-Hydroxybutyric_acid
METLIN IDNot Available
PubChem Compound440864
PDB IDNot Available
ChEBI ID50613
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00000003
Good Scents IDNot Available
References
Synthesis ReferenceCarlier, J. P.; Henry, C.; Lorin, V.; Rouffignat, K. Conversion of DL-threonine, D-threonine and 2-oxobutyrate into propionate and 2-hydroxybutyrate by Fusobacterium species. Letters in Applied Microbiology (1997), 25(5), 371-374.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Sweatman BC, Farrant RD, Holmes E, Ghauri FY, Nicholson JK, Lindon JC: 600 MHz 1H-NMR spectroscopy of human cerebrospinal fluid: effects of sample manipulation and assignment of resonances. J Pharm Biomed Anal. 1993 Aug;11(8):651-64. [PubMed:8257730 ]
  2. Landaas S, Pettersen JE: Clinical conditions associated with urinary excretion of 2-hydroxybutyric acid. Scand J Clin Lab Invest. 1975 May;35(3):259-66. [PubMed:168632 ]
  3. 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 ]
  4. Li X, Xu Z, Lu X, Yang X, Yin P, Kong H, Yu Y, Xu G: Comprehensive two-dimensional gas chromatography/time-of-flight mass spectrometry for metabonomics: Biomarker discovery for diabetes mellitus. Anal Chim Acta. 2009 Feb 9;633(2):257-62. doi: 10.1016/j.aca.2008.11.058. Epub 2008 Dec 3. [PubMed:19166731 ]
  5. Gall WE, Beebe K, Lawton KA, Adam KP, Mitchell MW, Nakhle PJ, Ryals JA, Milburn MV, Nannipieri M, Camastra S, Natali A, Ferrannini E: alpha-hydroxybutyrate is an early biomarker of insulin resistance and glucose intolerance in a nondiabetic population. PLoS One. 2010 May 28;5(5):e10883. doi: 10.1371/journal.pone.0010883. [PubMed:20526369 ]
  6. 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 ]
  7. (). Aldrich Library of FT-IR Spectra, 1st edn., 1985, 1, 548D. .
  8. (). Mori, K. et al., Tetrahedron, 1979, 35, 1601. .
  9. (). Levene, P.A. et al., J. Biol. Chem., 1941, 141, 391. .
  10. (). Duvillier, E., Ann. Chim. Phys., 1879, 17, 532. .
  11. (). Chenault, H.K. et al., J.O.C., 1987, 52, 2608. .
  12. (). Aldrich Library of 13C and 1H FT NMR Spectra, 1992, 1, 854B. .
  13. (). Horn, D.H.S. et al., J.C.S., 1954, 1460. .
  14. (). Wong, C.H. et al., J.O.C., 1985, 50, 1992. .

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
Gene Name:
DLD
Uniprot ID:
P09622
Molecular weight:
54176.91
References
  1. Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]
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
2-Hydroxybutyric acid → 2-(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
2-Hydroxybutyric acid → 6-(1-carboxypropoxy)-3,4,5-trihydroxyoxane-2-carboxylic aciddetails
2-Hydroxybutyric acid → 3,4,5-trihydroxy-6-[(2-hydroxybutanoyl)oxy]oxane-2-carboxylic aciddetails
General function:
Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:
Displays an lactate dehydrogenase activity. Significantly increases the transcriptional activity of JUN, when overexpressed.
Gene Name:
LDHAL6A
Uniprot ID:
Q6ZMR3
Molecular weight:
36507.015
Reactions
2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:
Not Available
Gene Name:
LDHB
Uniprot ID:
P07195
Molecular weight:
36638.225
Reactions
2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:
Possible role in sperm motility.
Gene Name:
LDHC
Uniprot ID:
P07864
Molecular weight:
36310.965
Reactions
2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:
Not Available
Gene Name:
LDHA
Uniprot ID:
P00338
Molecular weight:
30204.975
Reactions
2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:
Not Available
Gene Name:
LDHAL6B
Uniprot ID:
Q9BYZ2
Molecular weight:
41942.53
Reactions
2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Iondetails