GABA metabolism (aka GHB) (WP4157)

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.
last edited


Yasminomar, Egonw, and Fehrhart

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Homo sapiens


Inborn Errors of Metabolism (IEM) Pathways Rare Diseases


Pathway Ontology: xenobiotic metabolic pathway neurotransmitter metabolic pathway gamma-aminobutyric acid metabolic pathway

Disease Ontology: succinic semialdehyde dehydrogenase deficiency


Label Type Compact Identifier
gamma-aminobutanoic acid (GABA) Metabolite chebi:59888
succinic acid Metabolite chebi:30031
D-2-hydroxyglutarate transhydrogenase Protein uniprot:Q8IWW8
4-butyrolactone Metabolite chebi:42639
ADH Protein wikidata:Q410754
Serum paraoxonase/lactonase 3 (b) Protein eccode:
GHB dehydrogenase Protein eccode:
D-2-hydroxyglutaric acid Metabolite chebi:15801
gamma-hydroxybutyric acid (GHB) Metabolite chebi:16724
1,4-butanediol Metabolite chebi:41189
3-oxo-4-hydroxybutyric acid Metabolite pubchem.compound:19793911
ALDH Protein eccode:
SSA reductase Protein uniprot:O43488
GABAT Protein uniprot:P80404
4-hydroxybutyraldehyde Metabolite pubchem.compound:93093
fatty acid beta-oxidation spiral Pathway wikipathways:WP143
succinic semialdehyde Metabolite chebi:57706
alpha-ketoglutarate Metabolite chebi:16810
Serum paraoxonase/lactonase 3 (c) Protein eccode:
lactamase Protein uniprot:P16444
TCA cycle Pathway wikipathways:WP78
glycolic acid Metabolite chebi:17497
3,4-dihydroxybutyric acid Metabolite chebi:86371
Serum paraoxonase/lactonase 3 (a) Protein eccode:
ADH Protein wikidata:Q410754
SSADH Protein uniprot:P51649
glutamine Metabolite chebi:28300
succinic semialdehyde Metabolite chebi:57706
glutamic acid Metabolite chebi:16015
GABAT Protein uniprot:P80404
uracil Metabolite chebi:17568
beta-alanine Metabolite chebi:16958
malonic semialdehyde Metabolite chebi:17960
4,5-dihydroxyhexanoic acid Metabolite hmdb:HMDB0240745
4-hydroxybutyric acid Metabolite chebi:30830
3-oxo-4-hydroxybutanoic Metabolite pubchem.compound:19793911
2-oxo-4-hydroxybutanoic Metabolite chebi:173437
3-hydroxypropionic Metabolite chebi:33404
2-ketoglutarate Metabolite chebi:16810
D-2-hydroxyglutarate Metabolite chebi:15801
Serum paraoxonase/lactonase 3 Protein uniprot:Q15166


  1. Struys EA, Verhoeven NM, Ten Brink HJ, Wickenhagen WV, Gibson KM, Jakobs C. Kinetic characterization of human hydroxyacid-oxoacid transhydrogenase: relevance to D-2-hydroxyglutaric and gamma-hydroxybutyric acidurias. J Inherit Metab Dis. 2005;28(6):921–30. PubMed Europe PMC Scholia
  2. Struys EA, Verhoeven NM, Jansen EEW, Ten Brink HJ, Gupta M, Burlingame TG, et al. Metabolism of gamma-hydroxybutyrate to d-2-hydroxyglutarate in mammals: further evidence for d-2-hydroxyglutarate transhydrogenase. Metabolism. 2006 Mar;55(3):353–8. PubMed Europe PMC Scholia
  3. PubMed Europe PMC Scholia