Methylmalonate-Semialdehyde Dehydrogenase [acylating], Mitochondrial (ALDH6A1) Antibody

Methylmalonate-Semialdehyde Dehydrogenase [acylating], Mitochondrial (ALDH6A1)

Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.

ALDH6A1 belongs to the aldehyde dehydrogenases family of proteins. This enzyme plays a role in the valine and pyrimidine catabolic pathways. This protein is a mitochondrial methylmalonate semialdehyde dehydrogenase, and 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.

PBS containing 0.09% sodium azide.

This product is for research use only.

MMSA_HUMAN