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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2014-10-29 20:41:48 UTC
HMDB IDHMDB00112
Secondary Accession NumbersNone
Metabolite Identification
Common NameGamma-Aminobutyric acid
DescriptionGamma-aminobutyric acid (GABA) is an inhibitory neurotransmitter found in the nervous systems of widely divergent species. It is the chief inhibitory neurotransmitter in the vertebrate central nervous system. In vertebrates, GABA acts at inhibitory synapses in the brain. GABA acts by binding to specific transmembrane receptors in the plasma membrane of both pre- and postsynaptic neurons. This binding causes the opening of ion channels to allow either the flow of negatively-charged chloride ions into the cell or positively-charged potassium ions out of the cell. This will typically result in a negative change in the transmembrane potential, usually causing hyperpolarization. Three general classes of GABA receptor are known. These include GABAA and GABAC ionotropic receptors, which are ion channels themselves, and GABAB metabotropic receptors, which are G protein-coupled receptors that open ion channels via intermediaries (G proteins). Neurons that produce GABA as their output are called GABAergic neurons, and have chiefly inhibitory action at receptors in the vertebrate. Medium Spiny Cells are a typical example of inhibitory CNS GABAergic cells. GABA exhibits excitatory actions in insects, mediating muscle activation at synapses between nerves and muscle cells and also the stimulation of certain glands. GABA has also been shown to have excitatory roles in the vertebrate, most notably in the developing cortex. Organisms synthesize GABA from glutamate using the enzyme L-glutamic acid decarboxylase and pyridoxal phosphate as a cofactor. It is worth noting that this involves converting the principal excitatory neurotransmitter (glutamate) into the principal inhibitory one (GABA). Drugs that act as agonists of GABA receptors (known as GABA analogues or GABAergic drugs) or increase the available amount of GABA typically have relaxing, anti-anxiety and anti-convulsive effects. (http://en.wikipedia.org/wiki/GABA) Doses of GABA 1 to 3 g orally also have been used effectively to raise the IQ of mentally retarded persons. GABA is found to be deficient in cerebrospinal fluid and brain in many studies of experimental and human epilepsy. Benzodiazepines (such as Valium) are useful in status epilepticus because they act on GABA receptors. GABA increases in the brain after administration of many seizure medications. Hence, GABA is clearly an antiepileptic nutrient. Inhibitors of GAM metabolism can also produce convulsions. Spasticity and involuntary movement syndromes, e.g., Parkinson's, Friedreich's ataxia, tardive dyskinesia, and Huntington's chorea are all marked by low GABA when amino acid levels are studied. Trials of 2 to 3 g of GABA given orally have been effective in various epilepsy and spasticity syndromes. Agents that elevate GABA also are useful in lowering hypertension. Three grams orally have been effective in control of blood pressure. GABA is decreased in various encephalopathies. GABA can reduce appetite and is decreased in hypoglycemics. GABA reduces blood sugar in diabetics. Chronic brain syndromes can also be marked by deficiency of GABA; GABA has many promising uses in therapy. Cerebrospinal fluid levels of GABA may be useful in diagnosing very serious diseases. Vitamin B6, manganese, taurine and lysine can increase both GABA synthesis and effects, while aspartic acid and glutamic acid probably inhibit GABA effects. (http://www.dcnutrition.com/AminoAcids/Detail.CFM)The brain's principal inhibitory neurotransmitter, GABA, along with serotonin and norepinephrine, is one of several neurotransmitters that appear to be involved in the pathogenesis of anxiety and mood disorders. There are two principal subtypes of postsynaptic GABA receptor complexes, the GABA-A and GABA-B receptor complexes. Activation of the GABA-B receptor by GABA causes neuronal membrane hyperpolarization and a resultant inhibition of neurotransmitter release. In addition to binding sites for GABA, the GABA-A receptor has binding sites for benzodiazepines, barbiturates, and neurosteroids. GABA-A receptors are coupled to chloride ion channels; activation of the receptor induces increased inward chloride ion flux, resulting in membrane hyperpolarization and neuronal inhibition. After release into the synapse, free GABA that does not bind to either the GABA-A or GABA-B receptor complexes can be taken up by neurons and glial cells. Four different membrane transporter proteins, known as GAT-1, GAT-2, GAT-3, and BGT-1, which differ in their distribution in the CNS, are believed to mediate the uptake of synaptic GABA into neurons and glial cells. The GABA-A receptor subtype regulates neuronal excitability and rapid changes in fear arousal, such as anxiety, panic, and the acute stress response. Drugs that stimulate GABA-A receptors, such as the benzodiazepines and barbiturates, have anxiolytic and anti-seizure effects via GABA-A-mediated reduction of neuronal excitability, which effectively raises the seizure threshold. In support of the anticonvulsant and anxiolytic effects of the GABA-A receptor are findings that GABA-A antagonists produce convulsions in animals and the demonstration that there is decreased GABA-A receptor binding in a positron emission tomography (PET) study of patients with panic disorder. Low plasma GABA has been reported in some depressed patients and, in fact, may be a useful trait marker for mood disorders. (The Role Of GABA In The Pathogenesis And Treatment Of Anxiety And Other Neuropsychiatric Disorders: http://www.vcu-cme.org/gaba/overview.html).
Structure
Thumb
Synonyms
  1. 3-Carboxypropylamine
  2. 4-Aminobutanoate
  3. 4-Aminobutanoic acid
  4. 4-Aminobutyrate
  5. 4-Aminobutyric acid
  6. Aminalon
  7. GABA
  8. Gaballon
  9. Gamarex
  10. gamma Aminobutyrate
  11. gamma Aminobutyric acid
  12. gamma-Aminobutyrate
  13. gamma-Aminobutyric acid
  14. Gammalon
  15. Gammalone
  16. Gammar
  17. Gammasol
  18. Mielogen
  19. Mielomade
  20. Omega-Aminobutyrate
  21. Omega-Aminobutyric acid
  22. Piperidate
  23. Piperidic acid
  24. Piperidinate
  25. Piperidinic acid
  26. w-Aminobutyrate
  27. w-Aminobutyric acid
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
KingdomOrganic Compounds
Super ClassAmino Acids, Peptides, and Analogues
ClassAmino Acids and Derivatives
Sub ClassGamma Amino Acids and Derivatives
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Amino fatty acids(KEGG)
  • Amino fatty acids(Lipidmaps)
  • Biogenic amines(KEGG)
  • Other amino acids(KEGG)
  • amino acid zwitterion(ChEBI)
Substituents
  • Carboxylic Acid
  • Primary Aliphatic Amine (Alkylamine)
Direct ParentGamma Amino Acids and Derivatives
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Alanine and aspartate metabolism
  • Component of Butanoate metabolism
  • Component of Glutamate metabolism
  • Component of Taurine and hypotaurine metabolism
  • Component of beta-Alanine metabolism
ApplicationNot Available
Cellular locations
  • Extracellular
  • Mitochondria
  • Lysosome
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point203 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility1300.0 mg/mLNot Available
LogP-3.17HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility365 g/LALOGPS
logP-3ALOGPS
logP-2.9ChemAxon
logS0.55ALOGPS
pKa (Strongest Acidic)4.53ChemAxon
pKa (Strongest Basic)10.22ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity25.46ChemAxon
Polarizability10.62ChemAxon
Spectra
SpectraGC-MSMS/MSLC-MS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
  • Lysosome
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue Location
  • Adrenal Cortex
  • Brain
  • Epidermis
  • Fibroblasts
  • Hippocampus
  • Kidney
  • Muscle
  • Nerve Cells
  • Neuron
  • Spleen
  • Testes
Pathways
NameSMPDB LinkKEGG Link
Glutamate MetabolismSMP00072map00250
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.110 +/- 0.023 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified1.0 (0.0-2.0) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.24 +/- 0.096 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.32 (0.2 -0.45) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.23 +/- 0.075 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.05 (0.00-0.10) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.093 (0.083-0.10) uMAdult (>18 years old)BothNormal details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothNormal details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothNormal details
SalivaDetected and Quantified>10 uMAdult (>18 years old)BothNormal details
SalivaDetected and Quantified1.23 +/- 0.57 uMAdult (>18 years old)FemaleNormal details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
UrineDetected and Quantified0.09 +/- 0.10 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified<0.461 umol/mmol creatinineAdult (>18 years old)BothNormal
    details
    UrineDetected and Quantified0.17 +/- 0.025 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
    UrineDetected and Quantified0.25 +/- 0.20 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
    UrineDetected and Quantified1.3 (0.0-2.8) umol/mmol creatinineNewborn (0-30 days old)BothNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified0.75 (0.00-1.50) umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified103.89 (72.43 – 169.51) umol/mmol creatinineAdult (>18 years old)BothNormal
      • Lorena Ivona ŞTEF...
    details
    UrineDetected and Quantified2.9 (2.7-3.3) umol/mmol creatinineAdult (>18 years old)BothNormal details
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified0.46 +/- 0.11 uMAdult (>18 years old)BothHepatic encephalopathy details
    BloodDetected and Quantified1.45 (0.00-2.9) uMChildren (1-13 years old)BothGABA transaminase deficiency details
    BloodDetected and Quantified1.84 +/- 0.215 uMElderly (>65 years old)BothAlzheimer's disease details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.13 +/- 0.043 uMAdult (>18 years old)BothSpinocerebellar degeneration details
    Cerebrospinal Fluid (CSF)Detected and Quantified1.5 +/- 1.1 uMAdult (>18 years old)Not SpecifiedEpilepsy details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.1 (0.063 - 0.179) uMChildren (1-13 years old)Not SpecifiedFebrile seizure details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.52 - 0.57 uMChildren (1-13 years old)Not SpecifiedEpilepsy details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.11 (0.067 - 0.176) uMChildren (1-13 years old)Not SpecifiedEpilepsy details
    Cerebrospinal Fluid (CSF)Detected and Quantified2.4 (0.00-4.8) uMChildren (1-13 years old)BothGABA transaminase deficiency details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.1 (0.061 - 0.171) uMChildren (1-13 years old)Both
    Meningitis
    details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.016 uMNot SpecifiedNot SpecifiedInfant with typical pyridoxine-dependent seizures details
    Cerebrospinal Fluid (CSF)Detected and Quantified4.0 +/- 4.2 uMAdult (>18 years old)BothEpilepsy details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.0002 +/- 0.000013 uMAdult (>18 years old)BothTuberculous meningitis details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.00013 +/- 0.00002 uMAdult (>18 years old)Both
    Meningitis
    details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothOral cancer details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)FemaleBreast cancer details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Not SpecifiedPancreatic cancer details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Not SpecifiedPeriodontal diseases details
    SalivaDetected and Quantified1.08 +/- 0.34 uMAdult (>18 years old)MaleAlzheimer's disease details
    SalivaDetected and Quantified1.78 +/- 0.50 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothOral squamous cell carcinoma (OSCC) details
    SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothOral leukoplakia (OLK) details
    SalivaDetected and Quantified5.46 +/- 5.62 uMAdult (>18 years old)BothLewy body disease details
    UrineDetected and Quantified3.0 (1.5-4.5) umol/mmol creatinineChildren (1-13 years old)BothHyper beta-alaninemia details
    UrineDetected and Quantified118.51 (63.24 – 247.72) umol/mmol creatinineAdult (>18 years old)BothType 1 diabetes Mellitus
      • Lorena Ivona ŞTEF...
    details
    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
    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
    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
    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
    Hyper beta-alaninemia
    1. MetaGene
    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
    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
    Gaba-transaminase deficiency
    1. MetaGene
    Associated OMIM IDs
    DrugBank IDDB02530
    DrugBank Metabolite IDNot Available
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB IDFDB008937
    KNApSAcK IDC00001337
    Chemspider ID116
    KEGG Compound IDC00334
    BioCyc ID4-AMINO-BUTYRATE
    BiGG ID34652
    Wikipedia LinkGABA
    NuGOwiki LinkHMDB00112
    Metagene LinkHMDB00112
    METLIN IDNot Available
    PubChem Compound119
    PDB IDABU
    ChEBI ID16865
    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
    1. Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. Pubmed: 12097436
    2. Zarnowska ED, Pearce RA, Saad AA, Perouansky M: The gamma-subunit governs the susceptibility of recombinant gamma-aminobutyric acid type A receptors to block by the nonimmobilizer 1,2-dichlorohexafluorocyclobutane (F6, 2N). Anesth Analg. 2005 Aug;101(2):401-6, table of contents. Pubmed: 16037152
    3. Levy LM, Levy-Reis I, Fujii M, Dalakas MC: Brain gamma-aminobutyric acid changes in stiff-person syndrome. Arch Neurol. 2005 Jun;62(6):970-4. Pubmed: 15956168
    4. Hasler G, Neumeister A, van der Veen JW, Tumonis T, Bain EE, Shen J, Drevets WC, Charney DS: Normal prefrontal gamma-aminobutyric acid levels in remitted depressed subjects determined by proton magnetic resonance spectroscopy. Biol Psychiatry. 2005 Dec 15;58(12):969-73. Epub 2005 Jul 25. Pubmed: 16043137
    5. Denda M, Inoue K, Inomata S, Denda S: gamma-Aminobutyric acid (A) receptor agonists accelerate cutaneous barrier recovery and prevent epidermal hyperplasia induced by barrier disruption. J Invest Dermatol. 2002 Nov;119(5):1041-7. Pubmed: 12445190
    6. Wiens SC, Trudeau VL: Thyroid hormone and gamma-aminobutyric acid (GABA) interactions in neuroendocrine systems. Comp Biochem Physiol A Mol Integr Physiol. 2006 Jul;144(3):332-44. Epub 2006 Mar 9. Pubmed: 16527506
    7. Choi C, Coupland NJ, Hanstock CC, Ogilvie CJ, Higgins AC, Gheorghiu D, Allen PS: Brain gamma-aminobutyric acid measurement by proton double-quantum filtering with selective J rewinding. Magn Reson Med. 2005 Aug;54(2):272-9. Pubmed: 16032672
    8. Metzeler K, Agoston A, Gratzl M: An Intrinsic gamma-aminobutyric acid (GABA)ergic system in the adrenal cortex: findings from human and rat adrenal glands and the NCI-H295R cell line. Endocrinology. 2004 May;145(5):2402-11. Epub 2004 Jan 15. Pubmed: 14726441
    9. Naini AB, Vontzalidou E, Cote LJ: Isocratic HPLC assay with electrochemical detection of free gamma-aminobutyric acid in cerebrospinal fluid. Clin Chem. 1993 Feb;39(2):247-50. Pubmed: 8432013
    10. Levy LM, Henkin RI: Brain gamma-aminobutyric acid levels are decreased in patients with phantageusia and phantosmia demonstrated by magnetic resonance spectroscopy. J Comput Assist Tomogr. 2004 Nov-Dec;28(6):721-7. Pubmed: 15538143
    11. 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
    12. 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
    13. 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
    14. Nicholson-Guthrie CS, Guthrie GD, Sutton GP, Baenziger JC: Urine GABA levels in ovarian cancer patients: elevated GABA in malignancy. Cancer Lett. 2001 Jan 10;162(1):27-30. Pubmed: 11121859
    15. Nisijima K, Ishiguro T: Cerebrospinal fluid levels of monoamine metabolites and gamma-aminobutyric acid in neuroleptic malignant syndrome. J Psychiatr Res. 1995 May-Jun;29(3):233-44. Pubmed: 7473299

    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 + L-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
    L-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
    L-Glutamic acid → Gamma-Aminobutyric acid + CO(2)details
    L-Glutamic acid → Gamma-Aminobutyric acid + Carbon dioxidedetails
    General function:
    Involved in carboxy-lyase activity
    Specific function:
    Catalyzes the production of GABA.
    Gene Name:
    GAD1
    Uniprot ID:
    Q99259
    Molecular weight:
    66896.065
    Reactions
    L-Glutamic acid → Gamma-Aminobutyric acid + CO(2)details
    L-Glutamic acid → Gamma-Aminobutyric acid + Carbon dioxidedetails
    General function:
    Involved in catalytic activity
    Specific function:
    Decarboxylates L-arginine to agmatine. Truncated splice isoforms probably lack activity.
    Gene Name:
    ADC
    Uniprot ID:
    Q96A70
    Molecular weight:
    49979.185
    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:
    GABRG2
    Uniprot ID:
    P18507
    Molecular weight:
    54161.8
    General function:
    Involved in G-protein coupled receptor activity
    Specific function:
    Isoform 1E function may be to regulate the availability of functional GABA-B-R1A/GABA-B-R2 heterodimers by competing for GABA-B-R2 dimerization. This could explain the observation that certain small molecule ligands exhibit differential affinity for central versus peripheral sites
    Gene Name:
    GABBR1
    Uniprot ID:
    Q9UBS5
    Molecular weight:
    108319.4
    General function:
    Involved in nucleotide binding
    Specific function:
    Catalyzes the attachment of glutamate to tRNA(Glu) in a two-step reaction: glutamate is first activated by ATP to form Glu-AMP and then transferred to the acceptor end of tRNA(Glu) (By similarity).
    Gene Name:
    EARS2
    Uniprot ID:
    Q5JPH6
    Molecular weight:
    58688.1
    General function:
    Involved in G-protein coupled receptor activity
    Specific function:
    Receptor for GABA. The activity of this receptor is mediated by G-proteins that inhibit adenylyl cyclase activity, stimulates phospholipase A2, activates potassium channels, inactivates voltage-dependent calcium-channels and modulates inositol phospholipids hydrolysis. Plays a critical role in the fine-tuning of inhibitory synaptic transmission. Pre-synaptic GABA-B-R inhibit neurotransmitter release by down-regulating high- voltage activated calcium channels, whereas postsynaptic GABA-B-R decrease neuronal excitability by activating a prominent inwardly rectifying potassium (Kir) conductance that underlies the late inhibitory postsynaptic potentials. Not only implicated in synaptic inhibition but also in hippocampal long-term potentiation, slow wave sleep, muscle relaxation and antinociception
    Gene Name:
    GABBR2
    Uniprot ID:
    O75899
    Molecular weight:
    105820.5
    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:
    GABRA1
    Uniprot ID:
    P14867
    Molecular weight:
    51801.4
    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:
    GABRA2
    Uniprot ID:
    P47869
    Molecular weight:
    51325.9
    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:
    GABRA3
    Uniprot ID:
    P34903
    Molecular weight:
    55164.1
    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:
    GABRA4
    Uniprot ID:
    P48169
    Molecular weight:
    61622.6
    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:
    GABRA5
    Uniprot ID:
    P31644
    Molecular weight:
    52145.6
    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:
    GABRA6
    Uniprot ID:
    Q16445
    Molecular weight:
    51023.7
    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
    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:
    GABRB2
    Uniprot ID:
    P47870
    Molecular weight:
    59149.9
    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:
    GABRB3
    Uniprot ID:
    P28472
    Molecular weight:
    54115.0
    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:
    GABRD
    Uniprot ID:
    O14764
    Molecular weight:
    50707.8
    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:
    GABRE
    Uniprot ID:
    P78334
    Molecular weight:
    57971.2
    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:
    GABRG1
    Uniprot ID:
    Q8N1C3
    Molecular weight:
    53594.5
    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:
    GABRG3
    Uniprot ID:
    Q99928
    Molecular weight:
    54288.2
    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. In the uterus, the function of the receptor appears to be related to tissue contractility. The binding of this pI subunit with other GABA(A) receptor subunits alters the sensitivity of recombinant receptors to modulatory agents such as pregnanolone
    Gene Name:
    GABRP
    Uniprot ID:
    O00591
    Molecular weight:
    50639.7
    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. Rho-1 GABA receptor could play a role in retinal neurotransmission
    Gene Name:
    GABRR1
    Uniprot ID:
    P24046
    Molecular weight:
    55882.9
    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. Rho-2 GABA receptor could play a role in retinal neurotransmission
    Gene Name:
    GABRR2
    Uniprot ID:
    P28476
    Molecular weight:
    56816.6
    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:
    GABRR3
    Uniprot ID:
    A8MPY1
    Molecular weight:
    54271.1
    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:
    GABRQ
    Uniprot ID:
    Q9UN88
    Molecular weight:
    72020.9
    General function:
    Involved in catalytic activity
    Specific function:
    Catalyzes the synthesis of carnosine and homocarnosine. Carnosine is synthesized more efficiently than homocarnosine.
    Gene Name:
    CARNS1
    Uniprot ID:
    A5YM72
    Molecular weight:
    101318.725
    Reactions
    Adenosine triphosphate + L-Histidine + Gamma-Aminobutyric acid → Adenosine monophosphate + Pyrophosphate + Homocarnosinedetails
    General function:
    Involved in ATPase binding
    Specific function:
    Involved in intra-Golgi traffic. Modulates intra-Golgi transport through coupling between NSF activity and SNAREs activation. It first stimulates the ATPase activity of NSF which in turn stimulates the association with GOSR1
    Gene Name:
    GABARAPL2
    Uniprot ID:
    P60520
    Molecular weight:
    13666.7
    General function:
    Involved in beta-tubulin binding
    Specific function:
    May play a role in intracellular transport of GABA(A) receptors and its interaction with the cytoskeleton
    Gene Name:
    GABARAP
    Uniprot ID:
    O95166
    Molecular weight:
    13917.9
    General function:
    Involved in beta-tubulin binding
    Specific function:
    Increases cell-surface expression of kappa-type opioid receptor through facilitating anterograde intracellular trafficking of the receptor
    Gene Name:
    GABARAPL1
    Uniprot ID:
    Q9H0R8
    Molecular weight:
    14044.0