" /> methylmalonate-semialdehyde dehydrogenase (acylating) - CISMeF





Preferred Label : methylmalonate-semialdehyde dehydrogenase (acylating);

MeSH 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.;

MeSH synonym : dehydrogenase, mmsa; Methylmalonic-Semialdehyde Dehydrogenase (Acylating); MMSDH; MMSA Dehydrogenase;

Registry Number MeSH : EC 1.2.1.27;

Is substance : O;

UNII : EC 1.2.1.27;

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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.

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02/05/2025


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