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| Cat.No | ACP00537 | Target Name | ABAT |
|---|---|---|---|
| Target Synonyms | GABA aminotransferase;GABA-AT;Gamma-amino-N-butyrate transaminase;GABA transaminase;GABA-T;L-AIBAT | Form | Liquid or Lyophilized powder |
| Expression System | E.coli | Expression Range | 29-500aa |
| Mol Weight | 79.9 kDa | Protein Length | Full Length of Mature Protein |
| Purity | Greater than 85% as determined by SDS-PAGE. | Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | P80404 |
|---|
Uniprot Id
P80404
Target Species
Human
Target Name
ABAT
Target Full Name
4-aminobutyrate aminotransferase, mitochondrial
Target 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.
Target Involvement
GABA transaminase deficiency (GABATD)
Target Subcellular Location
Mitochondrion matrix.
Target Protein Families
Class-III pyridoxal-phosphate-dependent aminotransferase family
Target Tissue Specificity
Liver > pancreas > brain > kidney > heart > placenta.
Target Research Area
Neuroscience
Target Synonyms
(S) 3 amino 2 methylpropionate transaminase ; (S)-3-amino-2-methylpropionate transaminase; 4 aminobutyrate aminotransferase; 4 aminobutyrate aminotransferase; mitochondrial; 4-aminobutyrate aminotransferase; ABAT; FLJ17813; FLJ30272; GABA aminotransferase; GABA AT; GABA T; GABA transaminase; GABA transferase; GABA-AT; GABA-T; GABAT; GABT_HUMAN; Gamma amino N butyrate transaminase; Gamma-amino-N-butyrate transaminase; hCG1984265; L AIBAT; L-AIBAT; LAIBAT; mitochondrial; NPD009
Target Background
4-aminobutyrate aminotransferase (ABAT) is responsible for catabolism of gamma-aminobutyric acid (GABA), an important, mostly inhibitory neurotransmitter in the central nervous system, into succinic semialdehyde. The active enzyme is a homodimer of 50-kD subunits complexed to pyridoxal-5-phosphate. The protein sequence is over 95% similar to the pig protein. GABA is estimated to be present in nearly one-third of human synapses. ABAT in liver and brain is controlled by 2 codominant alleles with a frequency in a Caucasian population of 0.56 and 0.44. The ABAT deficiency phenotype includes psychomotor retardation, hypotonia, hyperreflexia, lethargy, refractory seizures, and EEG abnormalities. Multiple alternatively spliced transcript variants encoding the same protein isoform have been found for this gene.
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