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3-hydroxyisobutyrate dehydrogenase (3-hydroxy-2-methylpropanoate:NAD(+) oxidoreductase, EC 1.1. 1.31) is a dimeric mitochondrial enzyme that catalyzes the NAD(+)-dependent, reversible oxidation of 3-hydroxyisobutyrate, an intermediate of valine catabolism, to methylmalonate semialdehyde.
3'-hydroxyisobutyrate dehydrogenase; 3-hydroxyisobutyrate dehydrogenase; 3-hydroxyisobutyrate dehydrogenase, mitochondrial; 6430402H10Rik; AI265272; Hibadh; NS5ATP1
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