4-Aminobutyrate Aminotransferase (ABAT), Polyclonal Antibody

GABAT; L-AIBAT; 4-Anobutyrate Tansaminase; (S)-3-amino-2-methylpropionate transaminase; Gamma-amino-N-butyrate transaminase

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

200ug/ml (lot specific)

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customer’s specifications, please inquire.

Small volumes of anti-ABAT antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (ELISA)

56,439 Da

4-aminobutyrate aminotransferase

4-aminobutyrate aminotransferase, mitochondrial

4-aminobutyrate aminotransferase, mitochondrial

GABAT; GABA-AT; GABA transaminase; GABA-T  [Similar Products]

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. [provided by RefSeq, Jul 2008]

ABAT: 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. Defects in ABAT are a cause of GABA transaminase deficiency (GABATD). The phenotype of this deficiency includes psychomotor retardation, hypotonia, hyperreflexia, lethargy, refractory seizures, and EEG abnormalities. Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family.

Protein type: Amino Acid Metabolism - alanine, aspartate and glutamate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - butanoate; Carbohydrate Metabolism - propanoate; EC 2.6.1.19; EC 2.6.1.22; Mitochondrial; Other Amino Acids Metabolism - beta-alanine; Transferase

Chromosomal Location of Human Ortholog: 16p13.2

Cellular Component: 4-aminobutyrate transaminase complex; mitochondrial matrix; mitochondrion

Molecular Function: 4-aminobutyrate transaminase activity; protein homodimerization activity; pyridoxal phosphate binding; succinate-semialdehyde dehydrogenase binding

Biological Process: behavioral response to cocaine; gamma-aminobutyric acid catabolic process

Disease: Gaba-transaminase Deficiency

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