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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-05-18 22:15:09 UTC
HMDB IDHMDB00710
Secondary Accession NumbersNone
Metabolite Identification
Common Name4-Hydroxybutyric acid
Description4-Hydroxybutyric acid (GHB) is a precursor and a metabolite of gamma-aminobutyric acid (GABA), it is anticipated that GHB acts as a central nervous. system (CNS)neuromodulator, mediating its effects through GABA and GHB-specific receptors, or by affecting dopamine transmission.(PMID: 16620539 ). Gamma-hydroxybutyric acid (GHB) occurs naturally in all. mammals, but its function remains unknown. GHB is labeled as an illegal drug in most countries,but also used as a legal drug (Xyrem) in patients with narcolepsy. In high doses, GHB inhibits the CNS, inducing sleep and inhibiting the respiratory drive. In lower doses, its euphoriant effect predominates (PMID: 17658710 ).
Structure
Thumb
Synonyms
ValueSource
3-Carboxypropoxy acidChEBI
4-Hydroxy-butyric acidChEBI
4-Hydroxyalkanoic acidChEBI
4-HydroxybutanoateChEBI
4-Hydroxybutanoic acidChEBI
4-Hydroxycarboxylic acidChEBI
Gamma Hydroxybutyric acidChEBI
gamma-Hydroxybutyric acidChEBI
GHBChEBI
Oxy-N-butyric acidChEBI
XyremChEBI
4-HydroxybutyrateGenerator
4-Hydroxy-butyrateGenerator
4-HydroxyalkanoateGenerator
4-HydroxycarboxylateGenerator
g HydroxybutyrateGenerator
g Hydroxybutyric acidGenerator
gamma HydroxybutyrateGenerator
γ hydroxybutyrateGenerator
γ hydroxybutyric acidGenerator
g-HydroxybutyrateGenerator
g-Hydroxybutyric acidGenerator
gamma-HydroxybutyrateGenerator
γ-hydroxybutyrateGenerator
γ-hydroxybutyric acidGenerator
Oxy-N-butyrateGenerator
4-Hydroxy-butanoateHMDB
4-Hydroxy-butanoic acidHMDB
4-Hydroxybutyrate sodiumHMDB
4-Hydroxybutyric acid monosodium saltHMDB
gamma-Hydroxy butyrateHMDB
gamma-Hydroxy sodium butyrateHMDB
gamma-Hydroxybutyrate sodiumHMDB
Hydroxybutyric acidHMDB
Monosodium saltHMDB
Oxybate sodiumHMDB
Sodium gamma-hydroxybutyrateHMDB
Sodium gamma-oxybutyrateHMDB
Sodium oxybateHMDB
Chemical FormulaC4H8O3
Average Molecular Weight104.1045
Monoisotopic Molecular Weight104.047344122
IUPAC Name4-hydroxybutanoic acid
Traditional Namegamma hydroxybutyric acid
CAS Registry Number591-81-1
SMILES
OCCCC(O)=O
InChI Identifier
InChI=1S/C4H8O3/c5-3-1-2-4(6)7/h5H,1-3H2,(H,6,7)
InChI KeyInChIKey=SJZRECIVHVDYJC-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as short-chain hydroxy acids and derivatives. These are hydroxy acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassHydroxy acids and derivatives
Sub ClassShort-chain hydroxy acids and derivatives
Direct ParentShort-chain hydroxy acids and derivatives
Alternative Parents
Substituents
  • Short-chain hydroxy acid
  • Fatty acyl
  • Fatty acid
  • Straight chain fatty acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Primary alcohol
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Microbial
BiofunctionNot Available
ApplicationNot Available
Cellular locationsNot Available
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility494.0 mg/mLALOGPS
logP-0.63ALOGPS
logP-0.51ChemAxon
logS0.68ALOGPS
pKa (Strongest Acidic)4.44ChemAxon
pKa (Strongest Basic)-2.4ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area57.53 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity23.8 m3·mol-1ChemAxon
Polarizability10.17 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-0159-2920000000-131f94186a93d0aa315dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000i-9100000000-80636ab7e3cb47d4d58fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-05n4-9000000000-71899633e269029ab7f9View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9000000000-910235f5bc92bcca7469View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0udi-8900000000-5890f0a527dd16d9ac75View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0k9i-9200000000-241210fedf9304168090View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4l-9000000000-21d09118dfcfcb093f8dView in MoNA
MSMass Spectrum (Electron Ionization)splash10-0006-9000000000-d55d971a361b41564681View in MoNA
Biological Properties
Cellular LocationsNot Available
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified48.0 (0.0-96.0) uMAdult (>18 years old)BothNormal
    • Tietz NW eds. Cli...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.98 +/- 1.17 uMAdult (>18 years old)BothNormal details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
    • Zerihun T. Dame, ...
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
UrineDetected and Quantified7.2 (2.7-22.3) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified3.3 (0.0-10.8) umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified20.002 +/- 7.257 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified1.6 (0.3-5.8) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.0 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0.4 (0.0-1.9) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.37 (0.0-0.9) umol/mmol creatinineAdult (>18 years old)Female
Normal
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified17 - 594 uMChildren (1-13 years old)Not SpecifiedSuccinic semialdehyde dehydrogenase deficiency details
UrineDetected and Quantified17.277 +/- 19.542 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified1.00 (0.00-2.00) umol/mmol creatinineAdult (>18 years old)BothGlutaric aciduria II details
UrineDetected and Quantified3865.0 (130.00-7600.00) umol/mmol creatinineChildren (1-13 years old)BothGlutaric Aciduria II details
Associated Disorders and Diseases
Disease References
Succinic semialdehyde dehydrogenase deficiency
  1. Ergezinger K, Jeschke R, Frauendienst-Egger G, Korall H, Gibson KM, Schuster VH: Monitoring of 4-hydroxybutyric acid levels in body fluids during vigabatrin treatment in succinic semialdehyde dehydrogenase deficiency. Ann Neurol. 2003 Nov;54(5):686-9. [14595661 ]
Glutaric acidemia type 2
  1. MetaGene [Link]
Glutaric aciduria II
  1. MetaGene [Link]
Associated OMIM IDs
  • 271980 (Succinic semialdehyde dehydrogenase deficiency)
  • 231680 (Glutaric aciduria II)
  • 231680 (Glutaric acidemia type 2)
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022196
KNApSAcK IDNot Available
Chemspider ID9984
KEGG Compound IDC00989
BioCyc ID4-HYDROXY-BUTYRATE
BiGG IDNot Available
Wikipedia Linkgamma-hydroxybutyric acid
NuGOwiki LinkHMDB00710
Metagene LinkHMDB00710
METLIN ID5678
PubChem Compound10413
PDB IDNot Available
ChEBI ID30830
References
Synthesis ReferenceTakigawa, Shinichiro; Araya, Shuzo. Process for the preparation of g-hydroxybutyric acid as a synthetic intermediate. Jpn. Kokai Tokkyo Koho (1988), 3 pp.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Brown GK, Cromby CH, Manning NJ, Pollitt RJ: Urinary organic acids in succinic semialdehyde dehydrogenase deficiency: evidence of alpha-oxidation of 4-hydroxybutyric acid, interaction of succinic semialdehyde with pyruvate dehydrogenase and possible secondary inhibition of mitochondrial beta-oxidation. J Inherit Metab Dis. 1987;10(4):367-75. [3126356 ]
  2. Gibson KM, Baumann C, Ogier H, Rossier E, Vollmer B, Jakobs C: Pre- and postnatal diagnosis of succinic semialdehyde dehydrogenase deficiency using enzyme and metabolite assays. J Inherit Metab Dis. 1994;17(6):732-7. [7707697 ]
  3. Gibson KM, Aramaki S, Sweetman L, Nyhan WL, DeVivo DC, Hodson AK, Jakobs C: Stable isotope dilution analysis of 4-hydroxybutyric acid: an accurate method for quantification in physiological fluids and the prenatal diagnosis of 4-hydroxybutyric aciduria. Biomed Environ Mass Spectrom. 1990 Feb;19(2):89-93. [2407302 ]
  4. Shinka T, Inoue Y, Ohse M, Ito A, Ohfu M, Hirose S, Kuhara T: Rapid and sensitive detection of urinary 4-hydroxybutyric acid and its related compounds by gas chromatography-mass spectrometry in a patient with succinic semialdehyde dehydrogenase deficiency. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Aug 25;776(1):57-63. [12127325 ]
  5. Ergezinger K, Jeschke R, Frauendienst-Egger G, Korall H, Gibson KM, Schuster VH: Monitoring of 4-hydroxybutyric acid levels in body fluids during vigabatrin treatment in succinic semialdehyde dehydrogenase deficiency. Ann Neurol. 2003 Nov;54(5):686-9. [14595661 ]
  6. LeBeau MA, Montgomery MA, Morris-Kukoski C, Schaff JE, Deakin A, Levine B: A comprehensive study on the variations in urinary concentrations of endogenous gamma-hydroxybutyrate (GHB). J Anal Toxicol. 2006 Mar;30(2):98-105. [16620539 ]
  7. Kankaanpaa A, Liukkonen R, Ariniemi K: Determination of gamma-hydroxybutyrate (GHB) and its precursors in blood and urine samples: a salting-out approach. Forensic Sci Int. 2007 Aug 6;170(2-3):133-8. Epub 2007 Jul 20. [17658710 ]

Enzymes

General function:
Energy production and conversion
Specific function:
Catalyzes the NADPH-dependent reduction of succinic semialdehyde to gamma-hydroxybutyrate. May have an important role in producing the neuromodulator gamma-hydroxybutyrate (GHB). Has broad substrate specificity. Has NADPH-dependent aldehyde reductase activity towards 2-carboxybenzaldehyde, 2-nitrobenzaldehyde and pyridine-2-aldehyde (in vitro). Can reduce 1,2-naphthoquinone and 9,10-phenanthrenequinone (in vitro). Can reduce the dialdehyde protein-binding form of aflatoxin B1 (AFB1) to the non-binding AFB1 dialcohol. May be involved in protection of liver against the toxic and carcinogenic effects of AFB1, a potent hepatocarcinogen.
Gene Name:
AKR7A2
Uniprot ID:
O43488
Molecular weight:
39588.65
Reactions
4-Hydroxybutyric acid + NADP → Succinic acid semialdehyde + NADPHdetails
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). D,L-3-hydroxyisobutyrate and L-3-hydroxybutyrate (L-3-OHB) are also substrates for HOT with 10-fold lower activities.
Gene Name:
ADHFE1
Uniprot ID:
Q8IWW8
Molecular weight:
50307.42
Reactions
4-Hydroxybutyric acid + Oxoglutaric acid → Succinic acid semialdehyde + D-2-Hydroxyglutaric aciddetails