ALDH5A1, Polyclonal Antibody

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

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

Polyclonal

IgG

Rabbit

This antibody is purified through a protein A column, followed by peptide affinity purification.

100 ul of antibody in PBS with 0.09% (W/V) sodium azide
Appearance: Colorless liquid

At -20 degree C
Shelf Life: 12 months

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.

Polyclonal Antibody to detect ALDH5A1 in human and mouse samples

Background: Aldehyde dehydrogenases (ALDHs) mediate the NADP+-dependent oxidation of aldehydes into acids and play an important 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 (aldehyde dehydrogenase 5 family, member A1), also known as SSDH or SSADH, is a 535 amino acid protein that localizes to the mitochondria and belongs to the aldehyde dehydrogenase family. Expressed in a variety of tissues, including liver, heart, lung, brain, kidney and placenta, ALDH5A1 is required for gamma-aminobutyric acid (GABA) recycling from the synaptic cleft. Mutations of ALDH5A1 lead to succinate semialdehyde dehydrogenase deficiency (SSADH deficiency) that is characterized by severe ataxia and by mildly retarded psychomotor development.

Antibodies & Supporting Tools; Antibodies for Metabolic Processes (NEW)Diabetes, Obesity & Metabolic Syndrome; Antibodies (NEW) Antibodies & Supporting Tools; Primary Antibodies (A-Z)

Western Blot (WB), Immunohistochemistry (IHC)

Western blot: ~1:1000, IHC: ~1:10-1:50.

58,653 Da

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; EC 1.2.1.24; Amino Acid Metabolism - alanine, aspartate and glutamate; Oxidoreductase

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; glutamine metabolic process; glucosylceramide 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

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