Aldehyde Dehydrogenase 5 A1, Monoclonal Antibody

ALDH5A1 Antibody; Succinate-semialdehyde dehydrogenase mitochondrial; Aldehyde dehydrogenase family 5 member A1; NAD(+)-dependent succinic semialdehyde dehydrogenase; ALDH5A1; SSADH; SSDH; Aldehyde Dehydrogenase 5 A1, Mouse Anti Human

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

Monoclonal

IgG2a heavy chain and k light chain.

PAT17F5AT

Mouse

Antibody was purified from mouse ascitic fluids by protein-A affinity chromatography.

Sterile filtered colorless solution.
1mg/ml containing PBS, pH-7.4, 10% Glycerol and 0.02% Sodium Azide.

12 months at -20 degree C. 1 month at 4 degree C.
For periods up to 1 month store at 4 degree C, for longer periods of time, store at -20 degree C. Prevent freeze thaw cycles.

Small volumes of anti-ALDH5A1 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.

ALDH5A1 is a mitochondrial NAD(+)-dependent succinic semialdehyde dehydrogenase, which is a member of the aldehyde dehydrogenase family of proteins. The ALDH5A1 protein functions as a mediator to the NADP+-dependent oxidation of aldehydes into acids and has an imperative role in the detoxification of alcohol-derived acetaldehyde, as well as in lipid peroxidation and in the metabolism of corticosteroids, biogenic amines and neurotransmitters. ALDH5A1 is expressed in various tissues, including the liver, heart, lung, brain, kidney and placenta. Deficiency in the ALDH5A1 enzyme, known as 4-hydroxybutyricaciduria, is a rare inborn error in the metabolism of the neurotransmitter 4-aminobutyric acid (GABA). In response to this defect, physiologic fluids from patients accumulate GHB, which is a compound with numerous neuromodulatory properties.

MONOCLONAL ANTIBODIES, ANTI-HUMAN ENZYMES

ELISA (EIA), Western Blot (WB), Flow Cytometry (FC/FACS), Immunocytochemistry (ICC), Immunofluorescence (IF)

58,653 Da

aldehyde dehydrogenase 5 family member A1

succinate-semialdehyde dehydrogenase, mitochondrial

Succinate-semialdehyde dehydrogenase, mitochondrial

SSADH  [Similar Products]

This protein belongs to the aldehyde dehydrogenase family of proteins. This gene encodes a mitochondrial NAD(+)-dependent succinic semialdehyde dehydrogenase. A deficiency of this enzyme, known as 4-hydroxybutyricaciduria, is a rare inborn error in the metabolism of the neurotransmitter 4-aminobutyric acid (GABA). In response to the defect, physiologic fluids from patients accumulate GHB, a compound with numerous neuromodulatory properties. Two transcript variants encoding distinct isoforms have been identified for this gene. [provided by RefSeq, Jul 2008]

ALDH5A1: Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA). Defects in ALDH5A1 are the cause of succinate semialdehyde dehydrogenase deficiency (SSADH deficiency). SSADH deficiency is a rare inborn error in the metabolism of 4-aminobutyric acid (GABA) which leads to accumulation of 4-hydroxybutyric acid in physiologic fluids of patients. The disease is characterized by severe ataxia and by mildly retarded psychomotor development. Belongs to the aldehyde dehydrogenase family.

Protein type: Amino Acid Metabolism - alanine, aspartate and glutamate; Carbohydrate Metabolism - butanoate; EC 1.2.1.24; Mitochondrial; Oxidoreductase

Chromosomal Location of Human Ortholog: 6p22.3

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: aldehyde dehydrogenase (NAD) activity; protein homodimerization activity; succinate-semialdehyde dehydrogenase [NAD(P)+] activity; succinate-semialdehyde dehydrogenase activity

Biological Process: acetate metabolic process; central nervous system development; galactosylceramide metabolic process; gamma-aminobutyric acid catabolic process; glucose metabolic process; glutamate metabolic process; glutamine metabolic process; glutathione metabolic process; glycerophospholipid metabolic process; neurotransmitter catabolic process; protein homotetramerization; short-chain fatty acid metabolic process; succinate metabolic process

Disease: Succinic Semialdehyde Dehydrogenase Deficiency

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