aldehyde dehydrogenase family 5, member A1 (Aldh5A1, NAD(+)-dependent succinic semialdehyde dehydrogenase, SSADH, SSDH, Succinate semialdehyde dehydrogenase, mitochondrial precursor)

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.\n\nApplications: \nSuitable for use in Western Blot. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1-2ug/ml\nOptimal dilutions to be determined by the researcher.\n\nPositive Control: \nMouse Liver Tissue Lysate\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20 degrees C. Aliquots are stable for at least 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

A1334-36M

Size

100ug

Applications

WB

Hosts

Rabbit

Reactivities

Hum, Mouse, Rat

Form

Supplied as a liquid in PBS, 0.02% sodium azide.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

References

1. Vasiliou V and Pappa A. Polymorphisms of human aldehyde dehydrogenases. Consequences for drug metabolism and disease. Pharmacology 2000; 61:192-8. 2. Hearl WG and Churchich JE. Interactions between4-aminobutyrate aminotransferase and succinic semialdehyde dehydrogenase, two mitochondrial enzymes. J. Biol. Chem. 1984; 259:11459-63. 3. Gibson KM, Sweetman L, Nyhan WL, et al. Succinic semialdehyde dehydrogenase deficiency: an inborn error of gamma-aminobutyric acid metabolism. Clin. Chim. Acta 1983; 133:33-42.

Additional Info

Recognizes human Aldh5A1. Species Crossreactivity: Mouse and rat.