Methylmalonate-Semialdehyde Dehydrogenase (Acylating)

Medical Dictionary -> Methylmalonate-Semialdehyde Dehydrogenase (Acylating)

Methylmalonate-Semialdehyde Dehydrogenase (Acylating)


An enzyme that plays a role in the VALINE; LEUCINE; and ISOLEUCINE catabolic pathways by catalyzing the oxidation of 2-methyl-3-oxopropanate to propanoyl-CoA using NAD+ as a coenzyme. Methylmalonate semialdehyde dehydrogenase deficiency is characterized by elevated BETA-ALANINE and 3-HYDROXYPROPIONIC ACID.


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