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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-04-16 16:59:21 UTC
HMDB IDHMDB0000710
Secondary Accession Numbers
  • HMDB0015507
  • HMDB00710
  • HMDB15507
Metabolite Identification
Common Name4-Hydroxybutyric acid
Description4-Hydroxybutyric acid (also known as gamma-hydroxybutyrate or GHB) is a precursor and a metabolite of gamma-aminobutyric acid (GABA). 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 ). GHB occurs naturally in all mammals, but its function remains unknown. GHB is labeled as an illegal drug in most countries, but it also is used as a legal drug (Xyrem) in patients with narcolepsy. It is used illegally (under the street names juice, liquid ecstasy, or G) as an intoxicant for increasing athletic performance and as a date rape drug. In high doses, GHB inhibits the CNS, inducing sleep and inhibiting the respiratory drive. In lower doses, its euphoriant effect predominates (PMID: 17658710 ). When present in sufficiently high levels, 4-hydroxybutyric acid can act as an acidogen, a neurotoxin, and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A neurotoxin is a compound that adversely affects neural cells and tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of 4-hydroxybutyric acid are associated with two inborn errors of metabolism: glutaric aciduria II and succinic semialdehyde dehydrogenase deficiency (SSADH). SSADH deficiency leads to a 30-fold increase of GHB and a 2-4 fold increase of GABA in the brains of patients with SSADH deficiency as compared to normal brain concentrations of the compounds. As an acidogen, 4-hydroxybutyric acid is an organic acid, and abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, kidney abnormalities, liver damage, seizures, coma, and possibly death. Many affected children with organic acidemias experience intellectual disability or delayed development. These are also the characteristic symptoms of the untreated IEMs mentioned above. Particularly for SSADH deficiency, the most common features observed include developmental delay, hypotonia, and intellectual disability. Nearly half of patients exhibit ataxia, seizures, behaviour problems, and hyporeflexia. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures. As a neurotoxin, GHB appears to affect both GABA (a neurotransmitter) signaling and glutamate signaling (another neurotransmitter). Glutamine metabolism may also play a role in the pathophysiology of excessive levels of GHB. High levels of GHB have been shown to depress both the NMDA and AMPA/kainite receptor-mediated functions and may also alter glutamatergic excitatory synaptic transmission as well.
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
Chemical FormulaC4H8O3
Average Molecular Weight104.1045
Monoisotopic Molecular Weight104.047344122
IUPAC NameNot Available
Traditional NameNot Available
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 KeySJZRECIVHVDYJC-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentHydroxy fatty acids
Alternative Parents
Substituents
  • Short-chain hydroxy acid
  • Hydroxy fatty acid
  • Straight chain fatty acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Primary alcohol
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Biological location:

Source:

Process

Naturally occurring process:

Role

Biological role:

Industrial application:

Indirect biological role:

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 g/LALOGPS
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-0159-2920000000-131f94186a93d0aa315dView in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0159-2920000000-131f94186a93d0aa315dView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-0910000000-7240955b6b16291cf793View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0a5c-9000000000-915d32c0dbd6e6d19a55View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00di-9610000000-52e5254f6eba257ad184View 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
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue LocationNot Available
PathwaysNot Available
NameSMPDB/PathwhizKEGG
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.200 +/- 1.2(0-3.2) uMInfant (0-1 year old)Both
Normal
details
BloodDetected and Quantified48.0 (0.0-96.0) uMAdult (>18 years old)BothNormal
    • Tietz NW eds. Cli...
details
Cerebrospinal Fluid (CSF)Detected and Quantified<2.600 uMChildren (1 - 13 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.98 +/- 1.17 uMAdult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Both
Normal
    • Zerihun T. Dame, ...
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
UrineDetected and Quantified7.2 (2.7-22.3) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified1.8-2.9 umol/mmol creatinineAdult (>18 years old)FemaleNormal 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 Quantified1.6-2.0 umol/mmol creatinineAdult (>18 years old)MaleNormal 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
UrineDetected and Quantified3.300 +/- 3.300 umol/mmol creatinineInfant (0-1 year old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified2110-2920 uMInfant (0-1 year old)Male
Succinic semialdehyde dehydrogenase deficiency
details
Cerebrospinal Fluid (CSF)Detected and Quantified17 - 594 uMChildren (1-13 years old)Not SpecifiedSuccinic semialdehyde dehydrogenase deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified1760-2200 uMInfant (0-1 year old)Male
Succinic semialdehyde dehydrogenase deficiency
details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
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
    • MetaGene: Metabol...
details
UrineDetected and Quantified3865.0 (130.00-7600.00) umol/mmol creatinineChildren (1-13 years old)BothGlutaric Aciduria II
    • MetaGene: Metabol...
details
UrineDetected and Quantified880-3630 umol/mmol creatinineInfant (0-1 year old)Male
Succinic semialdehyde dehydrogenase deficiency
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. [PubMed:14595661 ]
  2. 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. [PubMed:12127325 ]
Glutaric aciduria II
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Associated OMIM IDs
  • 271980 (Succinic semialdehyde dehydrogenase deficiency)
  • 231680 (Glutaric aciduria II)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022196
KNApSAcK IDNot Available
Chemspider ID9984
KEGG Compound IDC01991
BioCyc ID4-HYDROXY-BUTYRATE
BiGG IDNot Available
Wikipedia LinkOxybate
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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed:17658710 ]
  8. Hedner T, Hedner J, Iversen K, Wessberg P, Lundborg P: Gammahydroxybutyric acid: central biochemical and behavioral effects in neonatal rats. Pharmacol Biochem Behav. 1985 Aug;23(2):185-9. [PubMed:2997806 ]
  9. van Amsterdam JG, Brunt TM, McMaster MT, Niesink R, van Noorden MS, van den Brink W: [Cognitive impairment due to intensive use and overdoses of gammahydroxybutyric acid (GHB)]. Tijdschr Psychiatr. 2012;54(12):1001-10. [PubMed:23250641 ]
  10. Stock G, Heidt H, Buss J, Schlor KH: Sleep patterns in cat induced by gammahydroxybutyric acid. Electroencephalogr Clin Neurophysiol. 1978 Apr;44(4):523-7. [PubMed:76561 ]
  11. Anden NE, Stock G: Inhibitory effect of gammahydroxybutyric acid and gammaaminobutyric acid on the dopamine cells in the substantia nigra. Naunyn Schmiedebergs Arch Pharmacol. 1973;279(1):89-92. [PubMed:4356204 ]
  12. Gomes C, Flygt C, Henning M, Norin L, Svensson TH, Trolin G: Gammahydroxy butyric acid: cardiovascular effects in the rat. J Neural Transm. 1976;38(2):123-9. [PubMed:1271050 ]
  13. Pedraza C, Garcia FB, Navarro JF: Neurotoxic effects induced by gammahydroxybutyric acid (GHB) in male rats. Int J Neuropsychopharmacol. 2009 Oct;12(9):1165-77. doi: 10.1017/S1461145709000157. Epub 2009 Mar 17. [PubMed:19288974 ]
  14. Hedner T, Lundborg P: Effect of gammahydroxybutyric acid on catecholamine synthesis and utilization in the developing rat brain. J Neural Transm. 1982;54(1-2):19-28. [PubMed:6809892 ]
  15. Hedner T, Lundborg P: Effect of gammahydroxybutyric acid on serotonin synthesis, concentration and metabolism in the developing rat brain. J Neural Transm. 1983;57(1-2):39-48. [PubMed:6194255 ]
  16. Johansson B, Hardebo JE: Cerebrovascular permeability and cerebral blood flow in hypertension induced by gammahydroxybutyric acid. An experimental study in the rat. Acta Neurol Scand. 1982 May;65(5):448-57. [PubMed:7113657 ]
  17. Hedner T, Lundborg P: Neurochemical characteristics of cerebral catecholamine neurons during the postnatal development in the rat. Med Biol. 1981 Aug;59(4):212-23. [PubMed:6803074 ]
  18. Giorgi O, Rubio MC: Decreased 3H-L-quinuclidinyl benzilate binding and muscarine receptor subsensitivity after chronic gamma-butyrolactone treatment. Naunyn Schmiedebergs Arch Pharmacol. 1981 Dec;318(1):14-8. [PubMed:7329448 ]
  19. Anden NE, Wachtel H: Biochemical effects of baclofen (beta-parachlorophenyl-GABA) on the dopamine and the noradrenaline in the rat brain. Acta Pharmacol Toxicol (Copenh). 1977 Feb;40(2):310-20. [PubMed:576560 ]
  20. Stock G: Failure of anticholinergic drugs to antagonize the increase in dopamine seen after gammahydroxybutyric acid and axotomy. J Neural Transm. 1979;44(1-2):137-43. [PubMed:438801 ]
  21. Baumann KW, Kassell NF, Olin J, Yamada T: The effects of gammahydroxybutyric acid on canine cerebral blood flow and metabolism. J Neurosurg. 1982 Aug;57(2):197-202. [PubMed:7086512 ]
  22. Stock G, Magnusson T, Anden NE: Increase in brain dopamine after axotomy or treatment with gammahydroxybutyric acid due to elimination of the nerve impulse flow. Naunyn Schmiedebergs Arch Pharmacol. 1973;278(4):347-61. [PubMed:4269968 ]
  23. Vulliemoz S, Vanini G, Truffert A, Chizzolini C, Seeck M: Epilepsy and cerebellar ataxia associated with anti-glutamic acid decarboxylase antibodies. J Neurol Neurosurg Psychiatry. 2007 Feb;78(2):187-9. [PubMed:17229747 ]
  24. Gessa GL: [Guidelines for the drug therapy of alcoholism]. Recenti Prog Med. 1990 Mar;81(3):171-5. [PubMed:2359869 ]
  25. Anden NE, Magnusson T, Stock G: Effects of drugs influencing monoamine mechanisms on the increase in brain dopamine produced by axotomy or treatment with gammahydroxybutyric acid. Naunyn Schmiedebergs Arch Pharmacol. 1973;278(4):363-72. [PubMed:4269969 ]
  26. Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
  27. Watson AD: Thematic review series: systems biology approaches to metabolic and cardiovascular disorders. Lipidomics: a global approach to lipid analysis in biological systems. J Lipid Res. 2006 Oct;47(10):2101-11. Epub 2006 Aug 10. [PubMed:16902246 ]
  28. Sethi JK, Vidal-Puig AJ: Thematic review series: adipocyte biology. Adipose tissue function and plasticity orchestrate nutritional adaptation. J Lipid Res. 2007 Jun;48(6):1253-62. Epub 2007 Mar 20. [PubMed:17374880 ]
  29. Lingwood D, Simons K: Lipid rafts as a membrane-organizing principle. Science. 2010 Jan 1;327(5961):46-50. doi: 10.1126/science.1174621. [PubMed:20044567 ]
  30. Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.

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
4-Hydroxybutyric acid → 4-(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
4-Hydroxybutyric acid → 6-(3-carboxypropoxy)-3,4,5-trihydroxyoxane-2-carboxylic aciddetails
4-Hydroxybutyric acid → 3,4,5-trihydroxy-6-[(4-hydroxybutanoyl)oxy]oxane-2-carboxylic aciddetails
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
General function:
Involved in ion transport
Specific function:
GABA, the major inhibitory neurotransmitter in the vertebrate brain, mediates neuronal inhibition by binding to the GABA/benzodiazepine receptor and opening an integral chloride channel
Gene Name:
GABRB1
Uniprot ID:
P18505
Molecular weight:
54234.1
References
  1. Maitre M, Humbert JP, Kemmel V, Aunis D, Andriamampandry C: [A mechanism for gamma-hydroxybutyrate (GHB) as a drug and a substance of abuse]. Med Sci (Paris). 2005 Mar;21(3):284-9. [PubMed:15745703 ]

Transporters

General function:
Involved in transmembrane transport
Specific function:
Proton-linked monocarboxylate transporter. Catalyzes the rapid transport across the plasma membrane of many monocarboxylates such as lactate, pyruvate, branched-chain oxo acids derived from leucine, valine and isoleucine, and the ketone bodies acetoacetate, beta-hydroxybutyrate and acetate
Gene Name:
SLC16A3
Uniprot ID:
O15427
Molecular weight:
49468.9
References
  1. Manning Fox JE, Meredith D, Halestrap AP: Characterisation of human monocarboxylate transporter 4 substantiates its role in lactic acid efflux from skeletal muscle. J Physiol. 2000 Dec 1;529 Pt 2:285-93. [PubMed:11101640 ]
General function:
Involved in transmembrane transport
Specific function:
Proton-linked monocarboxylate transporter. Catalyzes the rapid transport across the plasma membrane of many monocarboxylates such as lactate, pyruvate, branched-chain oxo acids derived from leucine, valine and isoleucine, and the ketone bodies acetoacetate, beta-hydroxybutyrate and acetate
Gene Name:
SLC16A1
Uniprot ID:
P53985
Molecular weight:
53957.7
References
  1. Lin RY, Vera JC, Chaganti RS, Golde DW: Human monocarboxylate transporter 2 (MCT2) is a high affinity pyruvate transporter. J Biol Chem. 1998 Oct 30;273(44):28959-65. [PubMed:9786900 ]
General function:
Involved in transmembrane transport
Specific function:
Proton-linked monocarboxylate transporter. Catalyzes the rapid transport across the plasma membrane of many monocarboxylates such as lactate, pyruvate, branched-chain oxo acids derived from leucine, valine and isoleucine, and the ketone bodies acetoacetate, beta-hydroxybutyrate and acetate. MCT2 is a high affinity pyruvate transporter
Gene Name:
SLC16A7
Uniprot ID:
O60669
Molecular weight:
52185.7
References
  1. Lin RY, Vera JC, Chaganti RS, Golde DW: Human monocarboxylate transporter 2 (MCT2) is a high affinity pyruvate transporter. J Biol Chem. 1998 Oct 30;273(44):28959-65. [PubMed:9786900 ]