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Methylmalonate-Semialdehyde Dehydrogenase (Acylating)

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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.      Other names MMSDH; Dehydrogenase, MMSA; Methylmalonic-Semialdehyde Dehydrogenase (Acylatin; MMSA Dehydrogenase

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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