- Catalog number
- PB10019
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- 4-Aminobutyrate aminotransferase is a protein that in humans is encoded by the ABAT gene. 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 humans ynapses. 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, refractoryseizures, and EEG abnormalities. Multiple alternatively spliced transcript variants encoding the same protein isoform have been found for this gene.
- Synonyms
- 4-aminobutyrate aminotransferase, mitochondrial;2.6.1.19; (S) -3-amino-2-methylpropionate transaminase;2.6.1.22; GABA aminotransferase; GABA-AT; Gamma-amino-N-butyrate transaminase; GABA transaminase; GABA-T; L-AIBAT; ABAT; GABAT
- Gene Name
- ABAT
- Gene ID
- 18
- UniProt
- P80404
- Host
- Rabbit
- Reactivity
- Human, Mouse, Rat
- Cross Reactivity
- No cross-reactivity with other proteins.
- Immunogen
- E. coli-derived human ABAT recombinant protein (Position: K388-K500) . Human ABAT shares 93.9% and 94.5% amino acid (aa) sequence identity with mouse and rat ABAT, respectively.
- Clonality
- Polyclonal
- Tissue Specificity
- Liver > pancreas > brain > kidney > heart > placenta.
- Applications
- Flow Cytometry, IF, ICC, WB
- Field of Research
- Amino Acid Metabolism, Amino Acids, Cancer, Cancer Metabolism, Metabolic Signaling Pathways, Metabolism, Metabolism Processes, Mitochondrial, Mitochondrial Markers, Mitochondrial Metabolism, Neuroscience, Neurotransmitter, Pathways and Processes, Response To Hypoxia, Signal Transduction
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- 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.
- References & Citations
- 1. De Biase D, Barra D, Simmaco M, John RA, Bossa F (Jan 1995) . Primary structure and tissue distribution of human 4-aminobutyrate aminotransferase. European Journal of Biochemistry / FEBS 227 (1-2) : 476–80. 2. Medina-Kauwe LK, Tobin AJ, De Meirleir L, Jaeken J, Jakobs C, Nyhan WL, Gibson KM (Jun 1999) . 4-Aminobutyrate aminotransferase (GABA-transaminase) deficiency.Journal of Inherited Metabolic Disease 22 (4) : 414–27.
- Storage Conditions
- Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 56439 MW
- Observed Molecular Weight
- 54 kDa
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- 6
- Gene Name Synonym
- 4-aminobutyrate aminotransferase, mitochondrial
- Subcellular Location
- Mitochondrion matrix.
- Sequence Similarities
- Belongs to the transient receptor (TC 1.A.4) family. STrpC subfamily. TRPC4 sub-subfamily.
- Protein Name
- 4-aminobutyrate aminotransferase, mitochondrial
- Isotype
- Rabbit IgG
- Contents
- Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.