This protein belongs to the aldehyde dehydrogenases family of proteins. This enzyme plays a role in the valine and pyrimidine catabolic pathways. The product of this gene, a mitochondrial methylmalonate semialdehyde dehydrogenase, catalyzes the irreversible oxidative decarboxylation of malonate and methylmalonate semialdehydes to acetyl- and propionyl-CoA. Methylmalonate semialdehyde dehydrogenase deficiency is characterized by elevated beta-alanine, 3-hydroxypropionic acid, and both isomers of 3-amino and 3-hydroxyisobutyric acids in urine organic acids.

Titration of the ALDH6A1 antibody may be required due to differences in protocols and secondary/substrate sensitivity.

This ALDH6A1 antibody was produced from a mouse immunized with ALDH6A1recombinant protein.

Aliquot the ALDH6A1 antibody and store frozen at -20oC or colder.Avoid repeated freeze-thaw cycles.