Aldh5A1 Antibody

Aldh5A1 Antibody: Aldh5A1 is a member of the aldehyde dehydrogenase superfamily, a group of NAD (P) (+) -dependent enzymes that catalyze the oxidation of a wide spectrum of aliphatic and aromatic aldehydes. Aldehyde dehydrogenase enzymes are thought to play a major role in the detoxification of aldehydes generated by alcohol metabolism and lipid peroxidation. Aldh5A1 is a mitochondrial NAD (+) -dependent succinic semialdehyde dehydrogenase. A deficiency of this enzyme, known as 4-hydroxybutyricaciduria, results in a disorder of the neurotransmitter 4-aminobutyric acid (GABA) . Symptoms usually include static encephalopathy, associated with developmental delays, hypotonia, ataxia, speech defects, and seizures. At least two isoforms of Aldh5A1 are known to exist.

Aldh5A1 antibody can be stored at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Hearl WG and Churchich JE. Interactions between4-aminobutyrate aminotransferase and succinic semialdehyde dehydrogenase, two mitochondrial enzymes. J. Biol. Chem.1984; 259:11459-63.

Gibson KM, Sweetman L, Nyhan WL, et al. Succinic semialdehyde dehydrogenase deficiency: an inborn error of gamma-aminobutyric acid metabolism. Clin. Chim. Acta1983; 133:33-42.