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Recombinant Human 4-aminobutyrate aminotransferase, mitochondrial (ABAT)

ACP24651

Number
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High Purity LevelsPrecision and ReliabilityCustomization Options

Specifications


Cat.No ACP24651 Target NameABAT
FormLyophilized powderExpression SystemCustom Production. Please inquire and provide the desire expression system.
Expression Range29-500Protein LengthFull Length of Mature Protein
Purity>85% (SDS-PAGE)Storage Buffer5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Immunogen Information


Target SpeciesHumanUniprot IDP80404
Background Information
  • Uniprot Id

    P80404

  • Target Species

    Human

  • Target Name

    ABAT

  • Target Full Name

    4-aminobutyrate aminotransferase, mitochondrial

  • Target 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.

  • Target Involvement

    GABA transaminase deficiency (GABATD)

  • Target Subcellular Location

    Mitochondrion matrix.

  • Target Protein Families

    Class-III pyridoxal-phosphate-dependent aminotransferase family

  • Target Tissue Specificity

    Liver > pancreas > brain > kidney > heart > placenta.

  • Target Research Area

    Neuroscience

  • Target Synonyms

    (S) 3 amino 2 methylpropionate transaminase ; (S)-3-amino-2-methylpropionate transaminase; 4 aminobutyrate aminotransferase; 4 aminobutyrate aminotransferase; mitochondrial; 4-aminobutyrate aminotransferase; ABAT; FLJ17813; FLJ30272; GABA aminotransferase; GABA AT; GABA T; GABA transaminase; GABA transferase; GABA-AT; GABA-T; GABAT; GABT_HUMAN; Gamma amino N butyrate transaminase; Gamma-amino-N-butyrate transaminase; hCG1984265; L AIBAT; L-AIBAT; LAIBAT; mitochondrial; NPD009

  • Target Background

    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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