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
BRENDA
CATH/Gene3D
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
GenitoUrinary Development Molecular Anatomy Project
Human Protein Atlas
KEGG Enzyme
KEGG Gene
OMIM
Orphanet
RefSeq Nucleotide
RefSeq Protein
UniProtKB
Wikipedia
Enzyme Reaction
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
Cofactor Species Comments Reference
pyridoxal phosphate None

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Inhibitors
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

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 23/10/2017. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=2464.