ALDH6A1 also known as Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial. ALDH6A1 is a mitochondrial methylmalonate semialdehyde dehydrogenase that plays a role in the valine and pyrimidine catabolic pathways. This protein catalyzes the irreversible oxidative decarboxylation of malonate and methylmalonate semialdehydes to acetyl- and propionyl-CoA. ALDH6A1 deficiency is characterized by elevated beta-alanine, 3-hydroxypropionic acid, and both isomers of 3-amino and 3-hydroxyisobutyric acids in urine organic acids. Recombinant human ALDH6A1, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.