ALDH5A1, Polyclonal Antibody

Succinate-semialdehyde dehydrogenase; mitochondrial; Aldehyde dehydrogenase family 5 member A1; NAD(+)-dependent succinic semialdehyde dehydrogenase; ALDH5A1; SSADH

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

Polyclonal

Rabbit Ig

Rabbit

This ALDH5A1 antibody is generated from rabbits immunized with human ALDH5A1 recombinant protein.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.43 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to 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 belongs to the aldehyde dehydrogenase family of proteins. This protein functions as 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.

Metabolism; Neuroscience; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS)

WB~~1:1000

Western blot analysis of ALDH5A1 Antibody in WiDr cell line lysates (35ug/lane). ALDH5A1 (arrow) was detected using the purified Pab.

Formalin-fixed and paraffin-embedded brain tissue reacted with ALDH5A1 Antibody, which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.

ALDH5A1 Antibody flow cytometric analysis of WiDr cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.

57215

aldehyde dehydrogenase 5 family, member A1

succinate-semialdehyde dehydrogenase, mitochondrial

Succinate-semialdehyde dehydrogenase, mitochondrial

SSADH  [Similar Products]

SSDH_HUMAN

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

Chromosomal Location of Human Ortholog: 6p22

Cellular Component: mitochondrion; mitochondrial matrix

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

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

Disease: Succinic Semialdehyde Dehydrogenase Deficiency

Knerr,I., J. Inherit. Metab. Dis. 30 (3), 279-294 (2007) Blasi,P., J. Mol. Evol. 63 (1), 54-68 (2006) Plomin,R., Mol. Psychiatry 9 (6), 582-586 (2004)

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