GABA metabolism (aka GHB) (Homo sapiens)

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1, 2SSADHgamma-butyrolactoneADH3,4-dihydrobutyric acid succinic acid d-2-hydroxyglutarate transhydrogenaseSerum paraoxonase/lactonase 3 (a)fatty acid beta-oxidation spiral4-hydroxybutaldehyde glycolic acidSerum paraoxonase/lactonase 3 (b)Serum paraoxonase/lactonase 3 (c) SSA reductasegamma-aminobutanoic acid (GABA)3-oxo-4-hydroxybutyric acidTCA cyclelactamaseGHB dehydrogenasesuccinic semialdehyde 1,4-butanediol GABA transaminase ALDHgamma-hydroxybutanoic acid (GHB)2ADH2ALDHglutamine2succinic semialdehyde 22glutamic acid22GABAT222uracilbeta-alanine22malonic semialdehyde222224,5-dihydroxyhexanoic acid2GABAT4-hydroxybutyric acid223-oxo-4-hydroxybutanoic2-oxo-4-hydroxybutanoic223-hydroxypropionic22222-ketoglutarate2D-2-hydroxyglutarate222DiseaseCatalysisPathwayStimulationProteinMetaboliteConversionConnection to diseaseLegend


Gamma-Hydroxybutyric acid (GHB) is a naturally occurring neurotransmitter and a psychoactive drug. This compound is a precursor to GABA, glutamate, and glycine in certain brain areas, acts on the GHB receptor and is a weak agonist at the GABAB receptor.

GHB has been used in a medical setting as a general anesthetic and as a treatment for cataplexy, narcolepsy, and alcoholism. It is also used illegally as an intoxicant, to try to increase athletic performance, and as a date rape drug. GHB is also produced as a result of fermentation, and is found in small quantities in some beers and wines, beef and small citrus fruits. Succinic semialdehyde dehydrogenase deficiency is a disease that causes GHB to accumulate in the blood.

Part of this pathway was inspired by Chapter 5 of the book of Blau (ISBN 3642403360 (978-3642403361)), Fig. 5.3.

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Ontology Terms



  1. Struys EA, Verhoeven NM, Jansen EE, Ten Brink HJ, Gupta M, Burlingame TG, Quang LS, Maher T, Rinaldo P, Snead OC, Goodwin AK, Weerts EM, Brown PR, Murphy TC, Picklo MJ, Jakobs C, Gibson KM; ''Metabolism of gamma-hydroxybutyrate to d-2-hydroxyglutarate in mammals: further evidence for d-2-hydroxyglutarate transhydrogenase.''; Metabolism, 2006 PubMed Europe PMC Scholia
  2. Nenad Blau, Carlo Dionisi Vici, K Michael Gibson, Marinus Duran; ''Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases''; Springer (ISBN-13: 978-3642403361), 2014


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114138view10:42, 23 December 2020DeSlUpdated description with fig. number.
114137view10:41, 23 December 2020DeSlAdded legend, converted interaction to disease to graphical lines.
114048view12:12, 18 December 2020DeSlUpdated PW with additonal metabolites
114047view12:05, 18 December 2020DeSlAdded additional disease, including OMIM links
114045view11:42, 18 December 2020DeSlAdded book ref to description
114044view11:41, 18 December 2020DeSlAdded lit. ref of Blau to specific sections
114043view11:36, 18 December 2020DeSlUpdating pathway with information from chapter 5, ed. 4 of Blau book.
113892view03:33, 7 December 2020EgonwMarked a datanode as disease
102675view09:15, 17 January 2019DeSlModified description
102674view09:13, 17 January 2019DeSlChanged weird characters in comment of 4-hydroxybutaldehyde
102673view09:10, 17 January 2019DeSlChanged layout of disease node
94898view13:55, 12 October 2017DeSlModified description
94882view10:05, 11 October 2017DeSlOntology Term : 'gamma-aminobutyric acid metabolic pathway' added !
94881view10:04, 11 October 2017DeSlOntology Term : 'succinic semialdehyde dehydrogenase deficiency' added !
94880view10:04, 11 October 2017DeSlOntology Term : 'neurotransmitter metabolic pathway' added !
94879view10:03, 11 October 2017DeSlAdded wikidata as database source where applicable
94878view10:02, 11 October 2017DeSlAdded ID for succinic semialdehyde, removed unused metabolite node.
94877view10:00, 11 October 2017DeSlAdded wikidata as database source where applicable
94876view09:58, 11 October 2017DeSlAdded literature ref, added content to the PW from this ref.
94875view09:22, 11 October 2017DeSlOntology Term : 'xenobiotic metabolic pathway' added !
94874view09:18, 11 October 2017DeSlAdded wikidata as database source where applicable
94873view09:16, 11 October 2017DeSlModified description
94872view09:14, 11 October 2017DeSlNew pathway

External references


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NameTypeDatabase referenceComment
d-2-hydroxyglutarate transhydrogenaseProtein1.1.99.24 (Enzyme Nomenclature)
1,4-butanediol MetaboliteQ161521 (Wikidata)
3,4-dihydrobutyric acid Metabolite150929 (PubChem-compound)
3-oxo-4-hydroxybutyric acidMetabolite19793911 (PubChem-compound)
4,5-dihydroxyhexanoic acidMetaboliteQ27161948 (Wikidata)
4-hydroxybutaldehyde Metabolite93093 (PubChem-compound) a.k.a. gamma-Hydroxybutyraldehyde
4-hydroxybutyric acidMetabolite
ADHProteinQ410754 (Wikidata) alcohol dehydrogenase
ALDHProtein1.2.1.3 (Enzyme Nomenclature)
  • aldehyde dehydrogenase
  • aka succinic acid semialdehyde dehydrogenase
GABA transaminase ProteinQ21118182 (Wikidata)
GABATProteinGABA transaminase
GHB dehydrogenaseProtein1.1.1.61 (Enzyme Nomenclature)
SSA reductaseProteinQ21097418 (Wikidata)
Serum paraoxonase/lactonase 3 (a)Protein3.1.8.1 (Enzyme Nomenclature)
Serum paraoxonase/lactonase 3 (b)Protein3.1.1.81 (Enzyme Nomenclature)
Serum paraoxonase/lactonase 3 (c) Protein3.1.1.2 (Enzyme Nomenclature)
TCA cyclePathwayWP78 (WikiPathways) aka Krebs Cycle
fatty acid beta-oxidation spiralPathwayWP143 (WikiPathways)
gamma-aminobutanoic acid (GABA)MetaboliteQ210021 (Wikidata) a.k.a. gamma-Aminobutyric acid
gamma-butyrolactoneMetaboliteQ79739 (Wikidata)
gamma-hydroxybutanoic acid (GHB)MetaboliteQ207920 (Wikidata) a.k.a. gamma-hydroxybutanoic acid
glutamic acidMetabolite
glycolic acidMetaboliteQ409373 (Wikidata)
lactamaseProtein3.5.2.6 (Enzyme Nomenclature)
malonic semialdehydeMetabolite
succinic acidMetaboliteQ213050 (Wikidata) aka succinate
succinic semialdehyde MetaboliteQ4602542 (Wikidata)

Annotated Interactions

No annotated interactions

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