2-methylbutyryl-coenzyme a dehydrogenase deficiency

Catalysis of the reaction: NAD+ + 2-methyl-3-hydroxybutyryl-CoA = NADH + H+ + 2-methylaceto-acetyl-CoA.

Disease Alternative Name

2-methyl-3-hydroxy-butyryl coa dehydrogenase activity
2-methyl-3-hydroxybutyryl coenzyme a dehydrogenase activity
(2s
3s)-3-hydroxy-2-methylbutanoyl-coa:nad+ oxidoreductase activity
2-methyl-3-hydroxybutyryl-coa dehydrogenase activity
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