Protein: 4-aminobutyrate aminotransferase, mitochondrial (EC 2.6.1.19) ((S)-3-amino-2-methylpropionate transaminase) (EC 2.6.1.22) (GABA aminotransferase) (GABA-AT) (Gamma-amino-N-butyrate transaminase) (GABA transaminase) (GABA-T) (L-AIBAT) (P80404)
Organism: Homo sapiens (Human)
Function: Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine (By similarity).
Subcellular location: Mitochondrion matrix.
Sequence: MASMLLAQRLACSFQHSYRLLVPGSRHISQAAAKVDVEFDYDGPLMKTEVPGPRSQELMKQLNIIQNAEAVHFFCNYEESRGNYLVDVDGNRMLDLYSQISSVPIGYSHPALLKLIQQPQNASMFVNRPALGILPPENFVEKLRQSLLSVAPKGMSQLITMACGSCSNENALKTIFMWYRSKERGQRGFSQEELETCMINQAPGCPDYSILSFMGAFHGRTMGCLATTHSKAIHKIDIPSFDWPIAPFPRLKYPLEEFVKENQQEEARCLEEVEDLIVKYRKKKKTVAGIIVEPIQSEGGDNHASDDFFRKLRDIARKHGCAFLVDEVQTGGGCTGKFWAHEHWGLDDPADVMTFSKKMMTGGFFHKEEFRPNAPYRIFNTWLGDPSKNLLLAEVINIIKREDLLNNAAHAGKALLTGLLDLQARYPQFISRVRGRGTFCSFDTPDDSIRNKLILIARNKGVVLGGCGDKSIRFRPTLVFRDHHAHLFLNIFSDILADFK
InterPro domains: - IPR005814: Aminotransferase class-III (family) [6-499]
- IPR015421: Pyridoxal phosphate-dependent transferase, major domain (homologous_superfamily) [109-409]
- IPR049704: Aminotransferases class-III pyridoxal-phosphate attachment site (conserved_site) [323-362]
- IPR015424: Pyridoxal phosphate-dependent transferase (homologous_superfamily) [40-498]
- IPR015422: Pyridoxal phosphate-dependent transferase, small domain (homologous_superfamily) [48-489]
- IPR004631: 4-aminobutyrate aminotransferase, eukaryotic (family) [37-496]
Interaction partners: - Succinate-semialdehyde dehydrogenase, mitochondrial
- Glutamate decarboxylase 2
- Glutamate decarboxylase 1
- Methylmalonate-semialdehyde/malonate-semialdehyde dehydrogenase [acylating], mitochondrial
- Beta-ureidopropionase
- Alpha-aminoadipic semialdehyde dehydrogenase
- Acidic amino acid decarboxylase GADL1
- Aldehyde dehydrogenase X, mitochondrial
- Aldehyde dehydrogenase family 3 member A2
- Beta-Ala-His dipeptidase
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0036094 (small molecule binding), GO:0016740 (transferase activity), GO:1901363 (heterocyclic compound binding), GO:0043167 (ion binding), GO:0097159 (organic cyclic compound binding), GO:0005515 (protein binding), GO:0019842 (vitamin binding), GO:0043177 (organic acid binding), GO:0046983 (protein dimerization activity), GO:0043168 (anion binding), GO:0042802 (identical protein binding), GO:0016769 (transferase activity, transferring nitrogenous groups), GO:0070279 (vitamin B6 binding), GO:0008483 (transaminase activity), GO:0042803 (protein homodimerization activity), GO:0031406 (carboxylic acid binding), GO:0030170 (pyridoxal phosphate binding), GO:0016597 (amino acid binding)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0065007 (biological regulation), GO:0065008 (regulation of biological quality), GO:0044237 (cellular metabolic process), GO:0071704 (organic substance metabolic process), GO:0044281 (small molecule metabolic process), GO:0009056 (catabolic process), GO:0044238 (primary metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0001505 (regulation of neurotransmitter levels), GO:0006520 (amino acid metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:0044282 (small molecule catabolic process), GO:1901575 (organic substance catabolic process), GO:0042133 (neurotransmitter metabolic process), GO:0006082 (organic acid metabolic process), GO:0044248 (cellular catabolic process), GO:0016054 (organic acid catabolic process), GO:1901565 (organonitrogen compound catabolic process), GO:0043436 (oxoacid metabolic process), GO:0009448 (gamma-aminobutyric acid metabolic process), GO:0009063 (amino acid catabolic process), GO:0019752 (carboxylic acid metabolic process), GO:0046395 (carboxylic acid catabolic process), GO:0032787 (monocarboxylic acid metabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0005739 (mitochondrion), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle)