Anti-ABAT Antibody Picoband® Fluoro488 Conjugated

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.

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

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.

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

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.

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.