The ABAT gene encodes 4-aminobutyric acid aminotransferase (GABA-T), an enzyme also known as gamma-aminobutyric acid transaminase. It catalyzes the transamination of GABA—the central nervous system’s primary inhibitory neurotransmitter—with alpha-ketoglutarate, producing succinic semialdehyde and glutamate to regulate GABA homeostasis in GABAergic signaling pathways.
Mutations in ABAT cause hereditary GABA transaminase deficiency, a rare neurometabolic disorder characterized by severe epilepsy, developmental delay, and hypotonia. Pharmacologically, GABA-T inhibitors like vigabatrin are approved for refractory epilepsy (e.g., infantile spasms) to boost synaptic GABA levels, though long-term use may risk visual field defects.
Recombinant Human 4-aminobutyrate aminotransferase, mitochondrial (ABAT) (CSB-EP001032HUe0)
Validated Data
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
ABAT Recombinant Monoclonal Antibody (CSB-RA242969A0HU)
Validated Data
IHC image of CSB-RA242969A0HU diluted at 1:100 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB.
ABAT Antibody (CSB-PA001032ESR1HU)
Validated Data
Western blot
All lanes: 4-aminobutyrate aminotransferase, mitochondrial antibody at 10μg/ml
Lane 1: Rat liver tissue
Lane 2: Mouse pancreatic tissue
Lane 3: Mouse kidney tissue
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 56 kDa
Observed band size: 56 kDa
Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA001032ESR1HU at dilution of 1:100
Immunohistochemistry of paraffin-embedded human placenta tissue using CSB-PA001032ESR1HU at dilution of 1:100
The following ABAT reagents supplied by CUSABIO are manufactured under a strict quality control system. Multiple applications have been validated and solid technical support is offered.
ABAT Antibodies for Homo sapiens (Human)
| Code | Product Name | Species Reactivity | Application |
|---|---|---|---|
| CSB-PA001032GA01HU | ABAT Antibody | Human,Mouse,Rat | ELISA,WB,IHC |
| CSB-PA001032ESR1HU | ABAT Antibody | Human, Mouse, Rat | ELISA, WB, IHC |
| CSB-RA242969A0HU | ABAT Recombinant Monoclonal Antibody | Human | ELISA, IHC |
ABAT Proteins for Homo sapiens (Human)
| Code | Product Name | Source |
|---|---|---|
| CSB-YP001032HU CSB-BP001032HU CSB-MP001032HU CSB-EP001032HU-B |
Recombinant Human 4-aminobutyrate aminotransferase, mitochondrial (ABAT) | Yeast Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
| CSB-EP001032HU | Recombinant Human 4-aminobutyrate aminotransferase, mitochondrial (ABAT) | E.coli |
| CSB-EP001032HUe0 | Recombinant Human 4-aminobutyrate aminotransferase, mitochondrial (ABAT) | E.coli |