ALDH6A1

(redirected from methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial)

ALDH6A1

A gene on chromosome 14q24.3 that encodes a mitochondrial methylmalonate semialdehyde dehydrogenase, an aldehyde dehydrogenase, which is involved in valine and pyrimidine catabolism. It causes the irreversible oxidative decarboxylation of malonate and methylmalonate semialdehydes to acetyl- and propionyl-CoA.