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3-metyl-2-oxobutanoátdehydrogenáza (lipoamid)

  1. Record numberd042942
    Date06.06.2025
    TypeM
    Topical term3-metyl-2-oxobutanoátdehydrogenáza (lipoamid)
    Other termEnglish (Pseudonym) 2-Oxoisovalerate Dehydrogenase (Lipoamide)
    Slovak (Pseudonym) 2-oxoizovalerátdehydrogenáza (lipoamid)
    See also(Skutočné meno) leucinóza
    UDCD08.811.682.657.350.760
    NoteA ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3.
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