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3-metyl-2-oxobutanoátdehydrogenáza (lipoamid)

  1. Subject h.3-metyl-2-oxobutanoátdehydrogenáza (lipoamid)
    Subject h.3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
    Entry terms2-oxoizovalerátdehydrogenáza (lipoamid)
    English X references2-Oxoisovalerate Dehydrogenase (Lipoamide)
    Scope note in EnglishA 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.
    See also reference (FX) in Slovak leucinóza
    See also reference (FX) in English Maple Syrup Urine Disease
    Links (1) - MeSH descriptor
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Number of the records: 1  

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