Anti-Zebrafish P5CS/ALDH18A1 Antibody

Lyophilized

At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.

Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

E.coli-derived zebrafish P5CS/ALDH18A1 recombinant protein (Position: R35-A204).

Delta-1-pyrroline-5-carboxylate synthetase (P5CS) is an enzyme that in humans is encoded by the ALDH18A1 gene. This gene is mapped to 10q24.1. This gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate,a critical step in the de novo biosynthesis of proline,ornithine and arginine. Mutations in this gene lead to hyperammonemia,hypoornithinemia,hypocitrullinemia,hypoargininemia and hypoprolinemia and may be associated with neurodegeneration,cataracts and connective tissue diseases. Alternatively spliced transcript variants,encoding different isoforms,have been described for this gene.