Names & Taxonomy

Uniprot ID:
P80404
Entry Name:
GABT_HUMAN
Status:
reviewed
Protein Names:
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)
Gene Names:
ABAT GABAT
Gene Names Primary:
ABAT
Organism:
Homo sapiens (Human)

Structure

Length:
500
Sequence:
MASMLLAQRLACSFQHSYRLLVPGSRHISQAAAKVDVEFDYDGPLMKTEVPGPRSQELMKQLNIIQNAEAVHFFCNYEESRGNYLVDVDGNRMLDLYSQISSVPIGYSHPALLKLIQQPQNASMFVNRPALGILPPENFVEKLRQSLLSVAPKGMSQLITMACGSCSNENALKTIFMWYRSKERGQRGFSQEELETCMINQAPGCPDYSILSFMGAFHGRTMGCLATTHSKAIHKIDIPSFDWPIAPFPRLKYPLEEFVKENQQEEARCLEEVEDLIVKYRKKKKTVAGIIVEPIQSEGGDNHASDDFFRKLRDIARKHGCAFLVDEVQTGGGCTGKFWAHEHWGLDDPADVMTFSKKMMTGGFFHKEEFRPNAPYRIFNTWLGDPSKNLLLAEVINIIKREDLLNNAAHAGKALLTGLLDLQARYPQFISRVRGRGTFCSFDTPDDSIRNKLILIARNKGVVLGGCGDKSIRFRPTLVFRDHHAHLFLNIFSDILADFK
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion matrix.

Function

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.
Catalytic Activity:
4-aminobutanoate + 2-oxoglutarate = succinate semialdehyde + L-glutamate.; (S)-3-amino-2-methylpropanoate + 2-oxoglutarate = 2-methyl-3-oxopropanoate + L-glutamate.
Cofactor:
COFACTOR: Name=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326;
Cross Reference Drug Bank:
DB00160 DB00780 DB00119 DB00313 DB01080
Gene Ontology Go:
4-aminobutyrate transaminase complex
extracellular exosome
mitochondrial matrix
mitochondrion
neuron projection
presynapse
(S)-3-amino-2-methylpropionate transaminase activity
4-aminobutyrate transaminase activity
iron-sulfur cluster binding
metal ion binding
protein homodimerization activity
pyridoxal phosphate binding
succinate-semialdehyde dehydrogenase binding
aging
behavioral response to cocaine
cerebellum development
copulation
exploration behavior
gamma-aminobutyric acid biosynthetic process
gamma-aminobutyric acid catabolic process
locomotory behavior
negative regulation of blood pressure
negative regulation of dopamine secretion
negative regulation of gamma-aminobutyric acid secretion
negative regulation of platelet aggregation
neurotransmitter catabolic process
neurotransmitter secretion
positive regulation of aspartate secretion
positive regulation of dopamine metabolic process
positive regulation of heat generation
positive regulation of inhibitory postsynaptic potential
positive regulation of insulin secretion
positive regulation of prolactin secretion
positive regulation of uterine smooth muscle contraction
response to drug
response to ethanol
response to hypoxia
response to iron ion
response to nicotine
synaptic transmission
Gene Ontology Biological Process:
aging
behavioral response to cocaine
cerebellum development
copulation
exploration behavior
gamma-aminobutyric acid biosynthetic process
gamma-aminobutyric acid catabolic process
locomotory behavior
negative regulation of blood pressure
negative regulation of dopamine secretion
negative regulation of gamma-aminobutyric acid secretion
negative regulation of platelet aggregation
neurotransmitter catabolic process
neurotransmitter secretion
positive regulation of aspartate secretion
positive regulation of dopamine metabolic process
positive regulation of heat generation
positive regulation of inhibitory postsynaptic potential
positive regulation of insulin secretion
positive regulation of prolactin secretion
positive regulation of uterine smooth muscle contraction
response to drug
response to ethanol
response to hypoxia
response to iron ion
response to nicotine
synaptic transmission
Gene Ontology Molecular Function:
(S)-3-amino-2-methylpropionate transaminase activity
4-aminobutyrate transaminase activity
iron-sulfur cluster binding
metal ion binding
protein homodimerization activity
pyridoxal phosphate binding
succinate-semialdehyde dehydrogenase binding
Gene Ontology Cellular Component:
4-aminobutyrate transaminase complex
extracellular exosome
mitochondrial matrix
mitochondrion
neuron projection
presynapse
Keywords:
Acetylation
Aminotransferase
Complete proteome
Direct protein sequencing
Disease mutation
Disulfide bond
Iron
Iron-sulfur
Metal-binding
Mitochondrion
Neurotransmitter degradation
Polymorphism
Pyridoxal phosphate
Reference proteome
Transferase
Transit peptide

Publication

PubMed ID:
7721088 14702039 15489334 7851425 15528998 21269460 24275569 10407778