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) (P61922)
Organism: Mus musculus (Mouse)
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.
Subcellular location: Mitochondrion matrix {ECO:0000250}.
Sequence: MAFLLITRRLACSSQKNLHLFIPGSRYISQAAAKVDIEFDYDGPLMKTEVPGPRSKELMKQLNTIQNAEAVHFFCNYEESRGNYLVDVDGNRMLDLYSQISSVPIGYNHPALAKLVQQPQNASTFINRPALGILPPENFVDKLQESLMSVAPRGMSQLITMACGSCSNENAFKTIFMWYRSKERGQRGFSKEELETCMVNQSPGCPDYSILSFMGAFHGRTMGCLATTHSKAIHKIDIPSFDWPIAPFPRLKYPLEEFTTDNQQEEARCLEEVEDLIVKYRKKKRTVAGIIVEPIQSEGGDNHASDDFFRKLRDIARKHGCAFLVDEVQTGGGCTGKFWAHEHWGLDDPADVMTFSKKMMTGGFFHKEEFRPSAPYRIFNTWLGDPSKNLLLAEVINIIKREDLLNNVARVGKTLLTGLLDLQAQYPQFISRVRGRGTFCSFDTPDEAIRNKLILIARNKGVVLGGCGDKSIRFRPTLVFRDHHAHLFLSIFSGILADFK
InterPro domains: - IPR005814: Aminotransferase class-III (family) [12-499]
- 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) [40-497]
- 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
- Methylmalonate-semialdehyde/malonate-semialdehyde dehydrogenase [acylating], mitochondrial
- Glutamate decarboxylase 1
- Glutamate decarboxylase 2
- Beta-ureidopropionase
- Aldehyde dehydrogenase X, mitochondrial
- Aldehyde dehydrogenase family 3 member A2
- Aldehyde dehydrogenase, mitochondrial
- Alpha-aminoadipic semialdehyde dehydrogenase
- 4-trimethylaminobutyraldehyde dehydrogenase
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0003824 (catalytic activity), GO:0016740 (transferase activity), GO:0016769 (transferase activity, transferring nitrogenous groups), GO:0008483 (transaminase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0065007 (biological regulation), GO:0048518 (positive regulation of biological process), GO:0050789 (regulation of biological process), GO:0065008 (regulation of biological quality), GO:0023056 (positive regulation of signaling), GO:0032879 (regulation of localization), GO:0050794 (regulation of cellular process), GO:0023051 (regulation of signaling), GO:0051050 (positive regulation of transport), GO:0048522 (positive regulation of cellular process), GO:1903530 (regulation of secretion by cell), GO:1903829 (positive regulation of protein localization), GO:0051049 (regulation of transport), GO:0051047 (positive regulation of secretion), GO:0046887 (positive regulation of hormone secretion), GO:0010646 (regulation of cell communication), GO:0060341 (regulation of cellular localization), GO:0010647 (positive regulation of cell communication), GO:0051222 (positive regulation of protein transport), GO:0010817 (regulation of hormone levels), GO:0046883 (regulation of hormone secretion), GO:1903532 (positive regulation of secretion by cell), GO:0051223 (regulation of protein transport), GO:0090276 (regulation of peptide hormone secretion), GO:0090087 (regulation of peptide transport), GO:1904951 (positive regulation of establishment of protein localization), GO:0070201 (regulation of establishment of protein localization), GO:0002793 (positive regulation of peptide secretion), GO:0051046 (regulation of secretion), GO:0090277 (positive regulation of peptide hormone secretion), GO:0032880 (regulation of protein localization), GO:0050708 (regulation of protein secretion), GO:0050714 (positive regulation of protein secretion), GO:0002791 (regulation of peptide secretion), GO:0032024 (positive regulation of insulin secretion), GO:0035774 (positive regulation of insulin secretion involved in cellular response to glucose stimulus), GO:0061178 (regulation of insulin secretion involved in cellular response to glucose stimulus), GO:0050796 (regulation of insulin secretion)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0042995 (cell projection), GO:0005737 (cytoplasm), GO:0031974 (membrane-enclosed lumen), GO:0005739 (mitochondrion), GO:0043233 (organelle lumen), GO:0120025 (plasma membrane bounded cell projection), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0043005 (neuron projection), GO:0043231 (intracellular membrane-bounded organelle), GO:0005759 (mitochondrial matrix), GO:0070013 (intracellular organelle lumen)