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metylmalonátsemialdehyddehydrogenáza (acylujúca)

  1. Subject h.metylmalonátsemialdehyddehydrogenáza (acylujúca)
    Subject h.Methylmalonate-Semialdehyde Dehydrogenase (Acylating)
    Entry termsmetylmalonát-semialdehyddehydrogenáza (acylujúca)
    Scope note in EnglishAn 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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