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 Edition 5, Chapter 24 (Fig.24.2) of the book of Blau (ISBN 9783030677268) (Ed.4 Chapter 5, Fig. 5.3).
d9da77aka gamma-aminobutyric acidAKA Glutamic acid decarboxylase, exists in two isoforms with molecular weights of 67 and 65 kDa (GAD67 and GAD65), encoded by two different genes (GAD1 and GAD2)aka succinatea77aka hydroxyacid-oxoacid transhydrogenase (HOT)b72Glutaminase has tissue-specific isoenzymes and 4 isoforms: the GLS gene encodes 2 types (active in kidney, and the GLS2 gene encodes 2 forms (active in liver)AKA Solerol, ring-structure, appears to ba a biomarker for SSADH deficiency [PMID:3126356]ce8alcohol dehydrogenase; Wikidata:Q410754aka NAD-dependent 4-hydroxybutyrate dehydrogenaseaka D-2-hydroxyglutarateaka 4-hydroxybutanoic acid, Gamma-HydroxybutyrateAKA 4-Acetamidobutanalaka 4-hydroxy-3-oxobutanoic acidaldehyde dehydrogenaseGABA transaminaseAKA 4-Acetamidobutyric acidaka 4-hydroxybutanalb72aka 2-ketoglutarate
aka 2-oxoglutarateAKA GAD67aka Krebs Cyclea77AKA Pyridoxal phosphate, pyridoxal 5'-phosphate, P5Palcohol dehydrogenase; Wikidata:Q410754Succinate-semialdehyde dehydrogenasea77a77AKA glutamatea77GABA transaminasea77a77a77a77AKA DHHA, open-structure, appears to ba a biomarker for SSADH deficiency [PMID:3126356]a77ce8a77aka 3-oxo-4-hydroxybutyric acid
aka 4-hydroxy-3-oxobutanoic acida77aka 2-oxo-4-hydroxybutyric acid
aka 4-hydroxy-2-oxobutanoic acida77aka 3-hydroxypropionic acida77a77D-2-hydroxyglutaric acida77AKA GAD65AKA Pyridoxal phosphate, pyridoxal 5'-phosphate, P5Paldehyde dehydrogenaseb72b72b72b72d9dd9dd1fparent compounds, not a specific reactiona77a77a77a77a77a77a77a77a77a77a77a77a77d9dGABA aminotransferase deficiencyDOID:0060174Diseasegamma-amino butyric acid metabolism disorderDOID:0060176Disease34244591PubMedRedefining differential roles of MAO-A in dopamine degradation and MAO-B in tonic GABA synthesisExp Mol Med.2021Hyun-U ChoSunpil KimJeongeun SimSeulkee YangHeeyoung AnMin-Ho NamDong-Pyo JangC Justin Leegamma-aminobutyric acid metabolic pathwayPW:0000412Pathway Ontology16435184PubMedKinetic characterization of human hydroxyacid-oxoacid transhydrogenase: relevance to D-2-hydroxyglutaric and gamma-hydroxybutyric acidurias.J Inherit Metab Dis2005Struys EAVerhoeven NMTen Brink HJWickenhagen WVGibson KMJakobs Cneurotransmitter metabolic pathwayPW:0000407Pathway Ontology16483879PubMedMetabolism of gamma-hydroxybutyrate to d-2-hydroxyglutarate in mammals: further evidence for d-2-hydroxyglutarate transhydrogenase.Metabolism2006Struys EAVerhoeven NMJansen EETen Brink HJGupta MBurlingame TGQuang LSMaher TRinaldo PSnead OCGoodwin AKWeerts EMBrown PRMurphy TCPicklo MJJakobs CGibson KMsuccinic semialdehyde dehydrogenase deficiencyDOID:0060175Disease9783642403361ISBNPhysician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic DiseasesSpringer (ISBN-13: 9783642403361)2014Nenad BlauCarlo Dionisi ViciK Michael GibsonMarinus Duranxenobiotic metabolic pathwayPW:0001229Pathway OntologyastrocyteCL:0000127Cell Type3126356PubMedUrinary 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-oxidationJ Inherit Metab Dis .1987G K BrownC H CrombyN J ManningR J Pollitt