4-aminobutyrate aminotransferase

Target id: 2464

Nomenclature: 4-aminobutyrate aminotransferase

Abbreviated Name: GABA-T

Family: GABA turnover

Annotation status:  image of a grey circle Awaiting annotation/under development. Please contact us if you can help with annotation.  » Email us

   GtoImmuPdb view: OFF :     Currently no data for 4-aminobutyrate aminotransferase in GtoImmuPdb

Gene and Protein Information
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human - 500 16p13.2 ABAT 4-aminobutyrate aminotransferase
Mouse - 500 16 A3 Abat 4-aminobutyrate aminotransferase
Rat - 500 10q12 Abat 4-aminobutyrate aminotransferase
Previous and Unofficial Names
GABA aminotransferase | GABA transaminase | GABA-AT | GABA-T | Gamma-amino-N-butyrate transaminase | L-AIBAT
Database Links
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
GenitoUrinary Development Molecular Anatomy Project
Human Protein Atlas
KEGG Enzyme
RefSeq Nucleotide
RefSeq Protein
Enzyme Reaction
EC Number: GABA + α-ketoglutaric acid = L-glutamic acid + 4-oxobutanoate
EC Number: (S)-3-amino-2-methylpropanoate + α-ketoglutaric acid = 2-methyl-3-oxopropanoate + L-glutamic acid
Cofactor Species Comments Reference
pyridoxal phosphate None

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Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Affinity Units Reference
CPP-115 Hs Inhibition 5.0 pKi 3
pKi 5.0 (Ki 9.7x10-6 M) [3]
vigabatrin Hs Irreversible inhibition 3.1 pKi 1,3
pKi 3.1 (Ki 8.5x10-4 M) [1,3]
4-acryloylphenol Hs Inhibition 5.3 pIC50 4
pIC50 5.3 (IC50 5.48x10-6 M) [4]
Clinically-Relevant Mutations and Pathophysiology
Disease:  GABA-transaminase deficiency
Synonyms: GABA aminotransferase deficiency [Disease Ontology: DOID:0060174]
Gamma-aminobutyric acid transaminase deficiency [Orphanet: ORPHA2066]
Disease Ontology: DOID:0060174
OMIM: 613163
Orphanet: ORPHA2066
References:  2


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1. Lippert B, Metcalf BW, Jung MJ, Casara P. (1977) 4-amino-hex-5-enoic acid, a selective catalytic inhibitor of 4-aminobutyric-acid aminotransferase in mammalian brain. Eur. J. Biochem.74 (3): 441-5. [PMID:856582]

2. Medina-Kauwe LK, Nyhan WL, Gibson KM, Tobin AJ. (1998) Identification of a familial mutation associated with GABA-transaminase deficiency disease. Neurobiol. Dis.5 (2): 89-96. [PMID:9746906]

3. Silverman RB. (2012) The 2011 E. B. Hershberg award for important discoveries in medicinally active substances: (1S,3S)-3-amino-4-difluoromethylenyl-1-cyclopentanoic acid (CPP-115), a GABA aminotransferase inactivator and new treatment for drug addiction and infantile spasms. J. Med. Chem.55 (2): 567-75. [PMID:22168767]

4. Tao YH, Yuan Z, Tang XQ, Xu HB, Yang XL. (2006) Inhibition of GABA shunt enzymes' activity by 4-hydroxybenzaldehyde derivatives. Bioorg. Med. Chem. Lett.16 (3): 592-5. [PMID:16290145]

How to cite this page

GABA turnover: 4-aminobutyrate aminotransferase. Last modified on 23/09/2015. Accessed on 20/03/2018. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=2464.