ENTRY       EC 1.3.99.10
NAME        Isovaleryl-CoA dehydrogenase
CLASS       Oxidoreductases
            Acting on the CH-CH group of donors
            With other acceptors
SYSNAME     3-Methylbutanoyl-CoA:(acceptor) oxidoreductase
REACTION    3-Methylbutanoyl-CoA + Acceptor = 3-Methylbut-2-enoyl-CoA +
            Reduced acceptor
SUBSTRATE   3-Methylbutanoyl-CoA
            n-Pentanoate
            Acceptor
PRODUCT     3-Methylbut-2-enoyl-CoA
            Reduced acceptor
COFACTOR    FAD
COMMENT     A flavoprotein; forms with electron-transferring flavoprotein
            and EC 1.5.5.1 a system reducing ubiquinone. n-Pentanoate can
            act as donor. Not identical with EC 1.3.99.2, 1.3.99.3 or
            1.3.99.12.
PATHWAY     PATH: MAP00280  Valine, leucine and isoleucine degradation
DISEASE     MIM: 243500  Isovaleryl Coenzyme A dehydrogenase;
                         Isovalericacidemia (3)
MOTIF       PS: PS00072  [GAC]-[LIVM]-[ST]-E-x(2)-[GSAN]-G-S-D-x(2)-[GSA]
            PS: PS00073  [QE]-x(2)-G-[GS]-x-G-[LIVMFY]-x(2)-[DEN]-x(4)-[KR]-
                         x(3)-[DE]
GENES       HSA: IVD(Hs.77510)
DBLINKS     University of Geneva ENZYME DATA BANK: 1.3.99.10
            WIT (What Is There) Metabolic Reconstruction: 1.3.99.10
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