Methylmalonate-Semialdehyde Dehydrogenase (Acylating)

**Definition:** 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-hydropropionic acid.

**Tree numbers:** - D08.811.682.657.163.827

**Synonyms:** - MMSDH - Methylmalonic-Semialdehyde Dehydrogenase (Acylating) - MMSA Dehydrogenase