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Mitochondria Pyruvate decarboxylase isozyme 3 acetolactate synthase / acetohydroxybutanoate synthase acetohydroxybutanoate synthase / acetolactate synthase acetohydroxybutanoate synthase / acetolactate synthase Valine Transaminase dihydroxy acid dehydratase, mitochondrial acetohydroxy acid isomeroreductase Pyruvic acid H + CO 2 (S)-2- Acetolactate Pyruvic acid H + CO 2 Isovaleric acid L-Glutamic acid Oxoglutaric acid L-Valine (R)-2,3- Dihydroxy- isovalerate H 2 O H + NADPH NADP (R)-2,3- Dihydroxy- isovalerate Glycolysis and Pyruvate Dehydrogenase
Mitochondria PDC6 Unknown Unknown Unknown Unknown Unknown Unknown Pyruvic acid Hydrogen Ion Carbon dioxide (S)-2- Acetolactate Pyruvic acid Hydrogen Ion Carbon dioxide Isovaleric acid L-Glutamic acid Oxoglutaric acid L-Valine (R)-2,3- Dihydroxy- isovalerate Water Hydrogen Ion NADPH NADP (R)-2,3- Dihydroxy- isovalerate Glycolysis and Pyruvate Dehydrogenase