4-aminobutyrate transaminase
Oxford Dictionary of Biochemistry:
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4-aminobutyrate transaminase
abbr.: GABA-T; EC 2.6.1.19; other names: γ-amino-N-butyrate transaminase; GABA transaminase; β-alanine-oxoglutarate aminotransferase; an aminotransferase enzyme important in the catabolism of γ-amino-n-butyric acid (abbr.: GABA). It catalyses the reaction:
4-aminobutanoate (GABA) + 2-oxoglutarate =succinate semialdehyde + l-glutamate.
Pyridoxal phosphate is the coenzyme. The human enzyme is a homodimer present in the mitochondrial matrix. Deficiency of the enzyme results from a rare missense mutation and is accompanied by seizures and psychomotor retardation and elevated levels of GABA, β-alanine, and homocarnosine in plasma and cerebrospinal fluid.
| 4-amino-4′-chlorodiphenyl, 3′AMP, 3′-phosphoadenylyl sulfate | |
| 4-carboxyglutamic acid, 4-coumaric acid, 4-hydroxybutyric aciduria |
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4-aminobutyrate transaminase
| 4-aminobutyrate transaminase | |||||||
|---|---|---|---|---|---|---|---|
| Identifiers | |||||||
| EC number | 2.6.1.19 | ||||||
| CAS number | 9037-67-6 | ||||||
| Databases | |||||||
| IntEnz | IntEnz view | ||||||
| BRENDA | BRENDA entry | ||||||
| ExPASy | NiceZyme view | ||||||
| KEGG | KEGG entry | ||||||
| MetaCyc | metabolic pathway | ||||||
| PRIAM | profile | ||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||
| Gene Ontology | AmiGO / EGO | ||||||
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| 4-aminobutyrate transaminase | |
|---|---|
| Identifiers | |
| Symbol | ABAT |
| Entrez | 18 |
| HUGO | 23 |
| OMIM | 137150 |
| RefSeq | NM_020686 |
| UniProt | P80404 |
| Other data | |
| Locus | Chr. 16 p13.2 |
In enzymology, 4-aminobutyrate transaminase (EC 2.6.1.19), also called GABA transaminase or 4-aminobutyrate aminotransferase, is an enzyme that catalyzes the chemical reaction:
- 4-aminobutanoate + 2-oxoglutarate
succinate semialdehyde + L-glutamate
Thus, the two substrates of this enzyme are 4-aminobutanoate (GABA) and 2-oxoglutarate. The two products are succinate semialdehyde and L-glutamate.
This enzyme belongs to the family of transferases, specifically the transaminases, which transfer nitrogenous groups. The systematic name of this enzyme class is 4-aminobutanoate:2-oxoglutarate aminotransferase. This enzyme participates in 5 metabolic pathways: alanine and aspartate metabolism, glutamate metabolism, beta-alanine metabolism, propanoate metabolism, and butanoate metabolism. It employs one cofactor, pyridoxal phosphate.
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Contents
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Structural Studies
As of late 2007, 9 structures have been solved for this class of enzymes, with PDB accession codes 1OHV, 1OHW, 1OHY, 1SF2, 1SFF, 1SZK, 1SZS, 1SZU, and 2EO5.
Inhibitors
References
- Scott EM, Jakoby WB (1959). "Soluble gamma-aminobutyric-glutamic transaminase from Pseudomonas fluorescens". J. Biol. Chem. 234 (4): 932–936. PMID 13654294.
- Aurich H (1961). "Ueber die beta-Alanin-alpha-Ketoglutarat-Transaminase aus Neurospora crassa". Hoppe-Seyler's Z. Physiol. Chem. 326: 25–33.
- Schousboe A, Wu JY, Roberts E (1973). "Purification and characterization of the 4-aminobutyrate:2-ketoglutarate transaminase from mouse brain". Biochemistry 12 (15): 2868–73. doi:10.1021/bi00739a015. PMID 4719123.
External links
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Oxford Dictionary of Biochemistry
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