DB code: D00104

CATH domain 3.90.1150.10 : Aspartate Aminotransferase, domain 1
3.40.640.10 : Aspartate Aminotransferase; domain 2 Catalytic domain
E.C. 2.6.1.19 2.6.1.22
CSA 1gtx
M-CSA 1gtx
MACiE

CATH domain Related DB codes (homologues)
3.40.640.10 : Aspartate Aminotransferase; domain 2 D00085 D00092 D00101 D00102 D00103 D00107 D00108 D00109 D00255 D00257 D00258 D00265 D00269 D00515 M00031 D00279
3.90.1150.10 : Aspartate Aminotransferase, domain 1 D00085 D00092 D00101 D00102 D00103 D00107 D00108 D00109 D00255 D00257 D00258 D00265 D00269 D00515 M00031 D00279

Uniprot Enzyme Name
UniprotKB Protein name Synonyms RefSeq Pfam
P80147 4-aminobutyrate aminotransferase, mitochondrial
EC 2.6.1.19
Gamma-amino-N-butyrate transaminase
GABA transaminase
GABA-T
GABA aminotransferase
GABA-AT
L-AIBAT
L-AIBAT) ((S)-3-amino-2-methylpropionate transaminase
EC 2.6.1.22
NP_999428.1 (Protein)
NM_214263.1 (DNA/RNA sequence)
PF00202 (Aminotran_3)
[Graphical View]

KEGG enzyme name
4-aminobutyrate transaminase
(EC 2.6.1.19 )
beta-alanine-oxoglutarate transaminase
(EC 2.6.1.19 )
aminobutyrate aminotransferase
(EC 2.6.1.19 )
beta-alanine aminotransferase
(EC 2.6.1.19 )
beta-alanine-oxoglutarate aminotransferase
(EC 2.6.1.19 )
gamma-aminobutyrate aminotransaminase
(EC 2.6.1.19 )
gamma-aminobutyrate transaminase
(EC 2.6.1.19 )
gamma-aminobutyrate-alpha-ketoglutarate aminotransferase
(EC 2.6.1.19 )
gamma-aminobutyrate-alpha-ketoglutarate transaminase
(EC 2.6.1.19 )
gamma-aminobutyrate:alpha-oxoglutarate aminotransferase
(EC 2.6.1.19 )
gamma-aminobutyric acid aminotransferase
(EC 2.6.1.19 )
gamma-aminobutyric acid pyruvate transaminase
(EC 2.6.1.19 )
gamma-aminobutyric acid transaminase
(EC 2.6.1.19 )
gamma-aminobutyric acid-alpha-ketoglutarate transaminase
(EC 2.6.1.19 )
gamma-aminobutyric acid-alpha-ketoglutaric acid aminotransferase
(EC 2.6.1.19 )
gamma-aminobutyric acid-2-oxoglutarate transaminase
(EC 2.6.1.19 )
gamma-aminobutyric transaminase
(EC 2.6.1.19 )
4-aminobutyrate aminotransferase
(EC 2.6.1.19 )
4-aminobutyrate-2-ketoglutarate aminotransferase
(EC 2.6.1.19 )
4-aminobutyrate-2-oxoglutarate aminotransferase
(EC 2.6.1.19 )
4-aminobutyrate-2-oxoglutarate transaminase
(EC 2.6.1.19 )
4-aminobutyric acid 2-ketoglutaric acid aminotransferase
(EC 2.6.1.19 )
4-aminobutyric acid aminotransferase
(EC 2.6.1.19 )
aminobutyrate aminotransferase
(EC 2.6.1.19 )
aminobutyrate transaminase
(EC 2.6.1.19 )
GABA aminotransferase
(EC 2.6.1.19 )
GABA transaminase
(EC 2.6.1.19 )
GABA transferase
(EC 2.6.1.19 )
GABA-alpha-ketoglutarate aminotransferase
(EC 2.6.1.19 )
GABA-alpha-ketoglutarate transaminase
(EC 2.6.1.19 )
GABA-alpha-ketoglutaric acid transaminase
(EC 2.6.1.19 )
GABA-alpha-oxoglutarate aminotransferase
(EC 2.6.1.19 )
GABA-2-oxoglutarate aminotransferase
(EC 2.6.1.19 )
GABA-2-oxoglutarate transaminase
(EC 2.6.1.19 )
GABA-oxoglutarate aminotransferase
(EC 2.6.1.19 )
GABA-oxoglutarate transaminase
(EC 2.6.1.19 )
glutamate-succinic semialdehyde transaminase
(EC 2.6.1.19 )
GabT
(EC 2.6.1.19 )
(S)-3-amino-2-methylpropionate transaminase
(EC 2.6.1.22 )
L-3-aminoisobutyrate transaminase
(EC 2.6.1.22 )
beta-aminobutyric transaminase
(EC 2.6.1.22 )
L-3-aminoisobutyric aminotransferase
(EC 2.6.1.22 )
beta-aminoisobutyrate-alpha-ketoglutarate transaminase
(EC 2.6.1.22 )

UniprotKB: Accession Number Entry name Activity Subunit Subcellular location Cofactor
P80147 GABT_PIG 4-aminobutanoate + 2-oxoglutarate = succinate semialdehyde + L-glutamate. (S)-3-amino-2-methylpropanoate + 2- oxoglutarate = 2-methyl-3-oxopropanoate + L-glutamate. Homodimer. Mitochondrion matrix. Pyridoxal phosphate.

KEGG Pathways
Map code Pathways E.C.
MAP00251 Glutamate metabolism 2.6.1.19
MAP00252 Alanine and aspartate metabolism 2.6.1.19
MAP00280 Valine, leucine and isoleucine degradation 2.6.1.22
MAP00410 beta-Alanine metabolism 2.6.1.19
MAP00640 Propanoate metabolism 2.6.1.19
MAP00650 Butanoate metabolism 2.6.1.19

Compound table
Cofactors Substrates Products Intermediates
KEGG-id C00018 C00334 C00026 C01205 C00022 C00232 C00025 C00349 C00041
E.C. 2.6.1.19
2.6.1.19
2.6.1.19
2.6.1.19
2.6.1.22
2.6.1.22
2.6.1.19
2.6.1.19
2.6.1.22
2.6.1.22
Compound Pyridoxal phosphate 4-Aminobutanoate 2-Oxoglutarate (R)-3-Amino-2-methylpropanoate Pyruvate Succinate semialdehyde L-Glutamate 2-Methyl-3-oxopropanoate L-alanine
Type aromatic ring (with nitrogen atoms),phosphate group/phosphate ion amino acids,lipid carbohydrate,carboxyl group amino acids carbohydrate,carboxyl group carbohydrate,carboxyl group amino acids,carboxyl group carbohydrate,carboxyl group amino acids
ChEBI 18405
16865
59888
30915
16320
57731
32816
16265
16015
16256
16977
57972
PubChem 1051
119
6992099
51
5459822
6971064
1060
1112
33032
44272391
88747398
296
5950
7311724
1gtxA01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1gtxB01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1gtxC01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1gtxD01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohvA01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohvB01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohvC01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohvD01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohwA01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohwB01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohwC01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohwD01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohyA01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohyB01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohyC01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohyD01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1gtxA02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1gtxB02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1gtxC02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1gtxD02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohvA02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohvB02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohvC02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohvD02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1ohwA02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Intermediate-analogue:PLP-VIG
1ohwB02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Intermediate-analogue:PLP-VIG
1ohwC02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Intermediate-analogue:PLP-VIG
1ohwD02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Intermediate-analogue:PLP-VIG
1ohyA02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Intermediate-analogue:PLP-GEG
1ohyB02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Intermediate-analogue:PLP-GEG
1ohyC02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Intermediate-analogue:PLP-GEG
1ohyD02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain Bound:PLP Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Intermediate-analogue:PLP-GEG

Reference for Active-site residues
resource references E.C.
literature [5] & [6]

Active-site residues
PDB Catalytic residues Cofactor-binding residues Modified residues Main-chain involved in catalysis Comment
1gtxA01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1gtxB01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1gtxC01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1gtxD01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohvA01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohvB01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohvC01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohvD01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohwA01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohwB01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohwC01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohwD01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohyA01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohyB01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohyC01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1ohyD01 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain
1gtxA02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1gtxB02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1gtxC02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1gtxD02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohvA02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohvB02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohvC02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohvD02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohwA02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohwB02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohwC02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohwD02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohyA02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohyB02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohyC02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)
1ohyD02 Pdbj logo s Rasmollogo id Rasmollogo chain Mmcif id Mmcif chain GLU 270;LYS 329 LYS 329(PLP binding)

References for Catalytic Mechanism
References Sections No. of steps in catalysis
[5]
p.8633
[6]
Scheme 2, Scheme 3

References
[1]
Resource
Comments
Medline ID
PubMed ID 6789830
Journal Biochem Biophys Res Commun
Year 1981
Volume 99
Pages 1333-40
Authors Kim DS, Churchich JE
Title 4-Aminobutyrate aminotransferase, the reaction of lysine residues connected with enzymatic activity.
Related PDB
Related UniProtKB
[2]
Resource
Comments
Medline ID
PubMed ID 7140743
Journal Eur J Biochem
Year 1982
Volume 126
Pages 507-11
Authors Churchich JE
Title 4-Aminobutyrate aminotransferase. Different susceptibility to inhibitors, microenvironment of the cofactor binding site and distance of the catalytic sites.
Related PDB
Related UniProtKB
[3]
Resource
Comments
Medline ID
PubMed ID 3780742
Journal Eur J Biochem
Year 1986
Volume 161
Pages 289-94
Authors Choi SY, Churchich JE
Title Biosynthesis of 4-aminobutyrate aminotransferase.
Related PDB
Related UniProtKB
[4]
Resource
Comments
Medline ID
PubMed ID 1901730
Journal Biochim Biophys Acta
Year 1991
Volume 1077
Pages 187-91
Authors Kim YT, Churchich JE
Title 4-Aminobutyrate aminotransferase: identification of lysine residues connected with catalytic activity.
Related PDB
Related UniProtKB
[5]
Resource
Comments X-RAY CRYSTALLOGRAPHY (3.0 ANGSTROMS)
Medline ID 99321499
PubMed ID 10393538
Journal Biochemistry
Year 1999
Volume 38
Pages 8628-34
Authors Storici P, Capitani G, De Biase D, Moser M, John RA, Jansonius JN, Schirmer T
Title Crystal structure of GABA-aminotransferase, a target for antiepileptic drug therapy.
Related PDB 1gtx
Related UniProtKB P80147
[6]
Resource
Comments
Medline ID
PubMed ID 11853435
Journal J Am Chem Soc
Year 2002
Volume 124
Pages 1620-4
Authors Choi S, Storici P, Schirmer T, Silverman RB
Title Design of a conformationally restricted analogue of the antiepilepsy drug Vigabatrin that directs its mechanism of inactivation of gamma-aminobutyric acid aminotransferase.
Related PDB
Related UniProtKB
[7]
Resource
Comments
Medline ID
PubMed ID 14534310
Journal J Biol Chem
Year 2004
Volume 279
Pages 363-73
Authors Storici P, De Biase D, Bossa F, Bruno S, Mozzarelli A, Peneff C, Silverman RB, Schirmer T
Title Structures of gamma-aminobutyric acid (GABA) aminotransferase, a pyridoxal 5'-phosphate, and [2Fe-2S] cluster-containing enzyme, complexed with gamma-ethynyl-GABA and with the antiepilepsy drug vigabatrin.
Related PDB 1ohv 1ohw 1ohy
Related UniProtKB

Comments

Created Updated
2004-03-17 2009-02-26