ABAT Antibody

Datasheet
Code CSB-PA001032GA01HU
Product Type Purified Rabbit Anti human PolyClonal Antibody
Size US$685
Uniprot No. P80404
Protocols ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol
Immunogen Human ABAT
Raised in Rabbit
Species Reactivity Human,Mouse,Rat
Tested Applications ELISA,WB,IHC
Storage Buffer PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Purification Method Antigen Affinity Purified
Isotype IgG
Alias 4-aminobutyrate aminotransferase;ABAT;FLJ17813;GABA-AT;GABAT;NPD009 ;
Immunogen Species Homo sapiens (Human)
Target Names ABAT
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Target Details 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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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.
Involvement in disease GABA transaminase deficiency (GABATD)
Subcellular Location Mitochondrion matrix
Protein Families Class-III pyridoxal-phosphate-dependent aminotransferase family
Tissue Specificity Liver > pancreas > brain > kidney > heart > placenta.
Database Links

HGNC: 23

OMIM: 137150

KEGG: hsa:18

STRING: 9606.ENSP00000268251

UniGene: Hs.336768

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