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4-aminobutyrate aminotransferase

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Target not currently curated in GtoImmuPdb

Target id: 2464

Nomenclature: 4-aminobutyrate aminotransferase

Abbreviated Name: GABA-T

Family: GABA turnover

Contents:

Gene and Protein Information Click here for help
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human - 500 16p13.2 ABAT 4-aminobutyrate aminotransferase
Mouse - 500 16 A1 Abat 4-aminobutyrate aminotransferase
Rat - 500 10q12 Abat 4-aminobutyrate aminotransferase
Previous and Unofficial Names Click here for help
GABA aminotransferase | GABA transaminase | GABA-AT | GABA-T | Gamma-amino-N-butyrate transaminase | L-AIBAT
Database Links Click here for help
Alphafold P80404 (Hs), P61922 (Mm), P50554 (Rn)
BRENDA 2.6.1.19, 2.6.1.22
CATH/Gene3D 3.90.1150.10, 3.40.640.10
ChEMBL Target CHEMBL2044 (Hs), CHEMBL4523258 (Mm), CHEMBL3148 (Rn)
DrugBank Target P80404 (Hs)
Ensembl Gene ENSG00000183044 (Hs), ENSMUSG00000057880 (Mm), ENSRNOG00000002636 (Rn)
Entrez Gene 18 (Hs), 268860 (Mm), 81632 (Rn)
Human Protein Atlas ENSG00000183044 (Hs)
KEGG Enzyme 2.6.1.19, 2.6.1.22
KEGG Gene hsa:18 (Hs), mmu:268860 (Mm), rno:81632 (Rn)
OMIM 137150 (Hs)
Orphanet ORPHA117617 (Hs)
Pharos P80404 (Hs)
RefSeq Nucleotide NM_001127448 (Hs), NM_000663 (Hs), NM_020686 (Hs), NM_172961 (Mm), NM_001170978 (Mm), NM_031003 (Rn)
RefSeq Protein NP_000654 (Hs), NP_065737 (Hs), NP_001120920 (Hs), NP_001164449 (Mm), NP_766549 (Mm), NP_112265 (Rn)
UniProtKB P80404 (Hs), P61922 (Mm), P50554 (Rn)
Wikipedia ABAT (Hs)
Enzyme Reaction Click here for help
EC Number: 2.6.1.19 GABA + α-ketoglutaric acid = L-glutamic acid + 4-oxobutanoate
EC Number: 2.6.1.22 (S)-3-amino-2-methylpropanoate + α-ketoglutaric acid = 2-methyl-3-oxopropanoate + L-glutamic acid
Cofactors Click here for help
Cofactor Species Comments Reference
pyridoxal 5-phosphate None

Download all structure-activity data for this target as a CSV file go icon to follow link

Inhibitors
Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Value Parameter Reference
CPP-115 Small molecule or natural product Primary target of this compound Hs Inhibition 5.0 pKi 3
⤷ pKi 5.0 (Ki 9.7x10-6 M) [3]
vigabatrin Small molecule or natural product Approved drug Primary target of this compound Click here for species-specific activity table Hs Irreversible inhibition 3.1 pKi 1,3
⤷ pKi 3.1 (Ki 8.5x10-4 M) [1,3]
4-acryloylphenol Small molecule or natural product Click here for species-specific activity table Hs Inhibition 5.3 pIC50 4
⤷ pIC50 5.3 (IC50 5.48x10-6 M) [4]
Clinically-Relevant Mutations and Pathophysiology Click here for help
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

References

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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 26/10/2025. IUPHAR/BPS Guide to PHARMACOLOGY, https://www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=2464.