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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:14:31 UTC
HMDB IDHMDB0000112
Secondary Accession Numbers
  • HMDB00112
Metabolite Identification
Common Namegamma-Aminobutyric acid
Description
Structure
Thumb
Synonyms
Chemical FormulaC4H9NO2
Average Molecular Weight103.1198
Monoisotopic Molecular Weight103.063328537
IUPAC Name4-aminobutanoic acid
Traditional Namegamma(amino)-butyric acid
CAS Registry Number56-12-2
SMILES
NCCCC(O)=O
InChI Identifier
InChI=1S/C4H9NO2/c5-3-1-2-4(6)7/h1-3,5H2,(H,6,7)
InChI KeyBTCSSZJGUNDROE-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as gamma amino acids and derivatives. These are amino acids having a (-NH2) group attached to the gamma carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentGamma amino acids and derivatives
Alternative Parents
Substituents
  • Gamma amino acid or derivatives
  • Amino fatty acid
  • Straight chain fatty acid
  • Fatty acid
  • Fatty acyl
  • Amino acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxide
  • Organopnictogen compound
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Organic oxygen compound
  • Carbonyl group
  • Organic nitrogen compound
  • Amine
  • Hydrocarbon derivative
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Disposition
Biological locationSource
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point203 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility1300 mg/mLNot Available
LogP-3.17HANSCH,C ET AL. (1995)
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
  • Lysosome
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Adrenal Cortex
  • Brain
  • Epidermis
  • Fibroblasts
  • Kidney
  • Neuron
  • Placenta
  • Skeletal Muscle
  • Spleen
  • Testis
Pathways
Normal Concentrations
Abnormal Concentrations
Associated Disorders and Diseases
Disease References
Alzheimer's disease
  1. Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed:17031479 ]
  2. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Hepatic encephalopathy
  1. Campollo O, MacGillivray BB, McIntyre N: [Association of plasma ammonia and GABA levels and the degree of hepatic encephalopathy]. Rev Invest Clin. 1992 Oct-Dec;44(4):483-90. [PubMed:1485027 ]
Gaba-transaminase deficiency
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Tuberculous meningitis
  1. Qureshi GA, Baig SM, Bednar I, Halawa A, Parvez SH: The neurochemical markers in cerebrospinal fluid to differentiate between aseptic and tuberculous meningitis. Neurochem Int. 1998 Feb;32(2):197-203. [PubMed:9542731 ]
Meningitis
  1. Qureshi GA, Baig SM, Bednar I, Halawa A, Parvez SH: The neurochemical markers in cerebrospinal fluid to differentiate between aseptic and tuberculous meningitis. Neurochem Int. 1998 Feb;32(2):197-203. [PubMed:9542731 ]
  2. Rating D, Siemes H, Loscher W: Low CSF GABA concentration in children with febrile convulsions, untreated epilepsy, and meningitis. J Neurol. 1983;230(4):217-25. [PubMed:6198481 ]
Epilepsy
  1. Spanaki MV, Siegel H, Kopylev L, Fazilat S, Dean A, Liow K, Ben-Menachem E, Gaillard WD, Theodore WH: The effect of vigabatrin (gamma-vinyl GABA) on cerebral blood flow and metabolism. Neurology. 1999 Oct 22;53(7):1518-22. [PubMed:10534261 ]
  2. Rating D, Siemes H, Loscher W: Low CSF GABA concentration in children with febrile convulsions, untreated epilepsy, and meningitis. J Neurol. 1983;230(4):217-25. [PubMed:6198481 ]
Febrile seizures
  1. Rating D, Siemes H, Loscher W: Low CSF GABA concentration in children with febrile convulsions, untreated epilepsy, and meningitis. J Neurol. 1983;230(4):217-25. [PubMed:6198481 ]
Pyridoxine-dependent epilepsy
  1. Kurlemann G, Ziegler R, Gruneberg M, Bomelburg T, Ullrich K, Palm DG: Disturbance of GABA metabolism in pyridoxine-dependent seizures. Neuropediatrics. 1992 Oct;23(5):257-9. [PubMed:1454145 ]
Hyperekplexia
  1. Dubowitz LM, Bouza H, Hird MF, Jaeken J: Low cerebrospinal fluid concentration of free gamma-aminobutyric acid in startle disease. Lancet. 1992 Jul 11;340(8811):80-1. [PubMed:1352015 ]
Irritable bowel syndrome
  1. Ponnusamy K, Choi JN, Kim J, Lee SY, Lee CH: Microbial community and metabolomic comparison of irritable bowel syndrome faeces. J Med Microbiol. 2011 Jun;60(Pt 6):817-27. doi: 10.1099/jmm.0.028126-0. Epub 2011 Feb 17. [PubMed:21330412 ]
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  2. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  3. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Crohn's disease
  1. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Ulcerative colitis
  1. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Perillyl alcohol administration for cancer treatment
  1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Pancreatic cancer
  1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Periodontal disease
  1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Frontotemporal dementia
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Lewy body disease
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Hyper beta-alaninemia
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Diabetes mellitus type 1
  1. (). Lorena Ivona ŞTEFAN, Alina NICOLESCU, Simona POPA, Maria MOŢA, Eugenia KOVACS and Calin DELEANU. 1H-NMR URINE METABOLIC PROFILING IN TYPE 1 DIABETES MELLITUS. Rev. Roum. Chim., 2010, 55(11-12), 1033-1037 . .
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
DrugBank IDDB02530
Phenol Explorer Compound IDNot Available
FooDB IDFDB030489
KNApSAcK IDC00001337
Chemspider ID116
KEGG Compound IDC00334
BioCyc ID4-AMINO-BUTYRATE
BiGG ID34652
Wikipedia LinkGamma-Aminobutyric_acid
METLIN IDNot Available
PubChem Compound119
PDB IDNot Available
ChEBI ID16865
Food Biomarker OntologyNot Available
VMH ID4ABUT
MarkerDB IDMDB00013416
Good Scents IDNot Available
References
Synthesis ReferenceMinoshima, Ryoichi. Preparation of gamma-aminobutyric acid with unripened beans. PCT Int. Appl. (2007), 25pp.
Material Safety Data Sheet (MSDS)Not Available
General References

Only showing the first 10 proteins. There are 38 proteins in total.

Enzymes

General function:
Involved in 4-aminobutyrate transaminase activity
Specific function:
Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine.
Gene Name:
ABAT
Uniprot ID:
P80404
Molecular weight:
56438.405
Reactions
gamma-Aminobutyric acid + Oxoglutaric acid → Succinic acid semialdehyde + Glutamic aciddetails
General function:
Involved in oxidoreductase activity
Specific function:
Converts gamma-trimethylaminobutyraldehyde into gamma-butyrobetaine. Catalyzes the irreversible oxidation of a broad range of aldehydes to the corresponding acids in an NAD-dependent reaction.
Gene Name:
ALDH9A1
Uniprot ID:
P49189
Molecular weight:
56291.485
Reactions
4-Aminobutyraldehyde + NAD + Water → gamma-Aminobutyric acid + NADHdetails
4-Aminobutyraldehyde + NADP + Water → gamma-Aminobutyric acid + NADPH + Hydrogen Iondetails
4-Aminobutyraldehyde + NAD + Water → gamma-Aminobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Multifunctional enzyme mediating important protective effects. Metabolizes betaine aldehyde to betaine, an important cellular osmolyte and methyl donor. Protects cells from oxidative stress by metabolizing a number of lipid peroxidation-derived aldehydes. Involved in lysine catabolism.
Gene Name:
ALDH7A1
Uniprot ID:
P49419
Molecular weight:
58486.74
Reactions
4-Aminobutyraldehyde + NADP + Water → gamma-Aminobutyric acid + NADPH + Hydrogen Iondetails
4-Aminobutyraldehyde + NAD + Water → gamma-Aminobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Recognizes as substrates free retinal and cellular retinol-binding protein-bound retinal. Seems to be the key enzyme in the formation of an RA gradient along the dorso-ventral axis during the early eye development and also in the development of the olfactory system (By similarity).
Gene Name:
ALDH1A3
Uniprot ID:
P47895
Molecular weight:
56107.995
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
ALDH2
Uniprot ID:
P05091
Molecular weight:
56380.93
Reactions
4-Aminobutyraldehyde + NADP + Water → gamma-Aminobutyric acid + NADPH + Hydrogen Iondetails
4-Aminobutyraldehyde + NAD + Water → gamma-Aminobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the oxidation of long-chain aliphatic aldehydes to fatty acids. Active on a variety of saturated and unsaturated aliphatic aldehydes between 6 and 24 carbons in length. Responsible for conversion of the sphingosine 1-phosphate (S1P) degradation product hexadecenal to hexadecenoic acid.
Gene Name:
ALDH3A2
Uniprot ID:
P51648
Molecular weight:
54847.36
Reactions
4-Aminobutyraldehyde + NADP + Water → gamma-Aminobutyric acid + NADPH + Hydrogen Iondetails
4-Aminobutyraldehyde + NAD + Water → gamma-Aminobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
ALDHs play a major role in the detoxification of alcohol-derived acetaldehyde. They are involved in the metabolism of corticosteroids, biogenic amines, neurotransmitters, and lipid peroxidation.
Gene Name:
ALDH1B1
Uniprot ID:
P30837
Molecular weight:
57248.96
Reactions
4-Aminobutyraldehyde + NADP + Water → gamma-Aminobutyric acid + NADPH + Hydrogen Iondetails
4-Aminobutyraldehyde + NAD + Water → gamma-Aminobutyric acid + NADH + Hydrogen Iondetails
General function:
Amino acid transport and metabolism
Specific function:
Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis.
Gene Name:
GATM
Uniprot ID:
P50440
Molecular weight:
48455.01
Reactions
L-Arginine + gamma-Aminobutyric acid → Ornithine + 4-Guanidinobutanoic aciddetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
General function:
Involved in glutamate-ammonia ligase activity
Specific function:
This enzyme has 2 functions: it catalyzes the production of glutamine and 4-aminobutanoate (gamma-aminobutyric acid, GABA), the latter in a pyridoxal phosphate-independent manner (By similarity). Essential for proliferation of fetal skin fibroblasts.
Gene Name:
GLUL
Uniprot ID:
P15104
Molecular weight:
42064.15
Reactions
Glutamic acid → gamma-Aminobutyric acid + CO(2)details
General function:
Involved in carboxy-lyase activity
Specific function:
Catalyzes the production of GABA.
Gene Name:
GAD2
Uniprot ID:
Q05329
Molecular weight:
65410.77
Reactions
Glutamic acid → gamma-Aminobutyric acid + CO(2)details
Glutamic acid → gamma-Aminobutyric acid + Carbon dioxidedetails

Only showing the first 10 proteins. There are 38 proteins in total.