Methylmalonate-Semialdehyde Dehydrogenase (Acylating) D050646

Chemicals and Drugs [D] » Enzymes and Coenzymes [D08] » Enzymes » Oxidoreductases » Oxidoreductases Acting on Aldehyde or Oxo Group Donors » Aldehyde Oxidoreductases » Methylmalonate-Semialdehyde Dehydrogenase (Acylating)

Description

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.   MeSH

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