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.
Alternative names and synonyms associated with ALDH6A1
aldehyde dehydrogenase 6 family member A1 (ALDH6A1) ELISA Kit
aldehyde dehydrogenase 6 family, member A1 (Aldh6a1) ELISA Kit
aldehyde dehydrogenase 6 family member A1 L homeolog (aldh6a1.L) ELISA Kit