Anti-ABAT antibody (AA34057)

$187.00


Alternative names 4-aminobutyrate aminotransferase, mitochondrial, EC 2.6.1.19,, S)-3-amino-2-methylpropionate transaminase, EC 2.6.1.22, GABA aminotransferase, GABA-AT, Gamma-amino-N-butyrate transaminase, GABA transaminase, GABA-T, L-AIBAT, ABAT, GABAT, GABT_HUMAN, P80404, 18
Source 500
Species Reactivity Human, Mouse, Rat
Applications IF/ICC, IHC, WB
Host Rabbit
Class Polyclonal
Conjugated Unconjugated


Description

Product name Anti-ABAT antibody (AA34057)
Catalog number AA34057
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names 4-aminobutyrate aminotransferase, mitochondrial antibody, EC 2.6.1.19 antibody, antibody, S)-3-amino-2-methylpropionate transaminase antibody, EC 2.6.1.22 antibody, GABA aminotransferase antibody, GABA-AT antibody, Gamma-amino-N-butyrate transaminase antibody, GABA transaminase antibody, GABA-T antibody, L-AIBAT antibody, ABAT antibody, GABAT antibody, GABT_HUMAN antibody


Biological Information

Immunogen Recombinant protein of human ABAT
Concentration 1 mg/ml
Isotype IgG
Application Notes WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Purification method Purified by affinity chromatography


Target

Target Name ABAT
SwissProt ID Link P80404
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.
Subcellular Location Mitochondrion matrix.
Involvement in Disease GABA transaminase deficiency: An enzymatic deficiency resulting in psychomotor retardation, hypotonia, hyperreflexia, lethargy, refractory seizures, and EEG abnormalities. The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity Liver > pancreas > brain > kidney > heart > placenta.
Subunit Structure Homodimer; disulfide-linked.
Sequence Similarities Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family.


Poduct Presentation

Form Liquid
Preservative 0.02% (w/v) sodium azide
Storage buffer PBS with 50% glycerol, pH 7.3


Storage and Shipping Information

Storage Store at -20 or -80 C. Avoid multiple freeze/thaw cycles.