2-methyl-3-hydroxybutyryl-coa dehydrogenase deficiency

Catalysis of the reaction: (S)-3-hydroxybutanoyl-CoA + NADP+ = 3-acetoacetyl-CoA + NADPH + H+.

Disease Alternative Name

beta-hydroxybutyryl coenzyme a dehydrogenase activity
beta-hydroxybutyryl-coa dehydrogenase activity
dehydrogenase
l-3-hydroxybutyryl coenzyme a (nicotinamide adenine dinucleotide phosphate)
bhbd activity
l-(+)-3-hydroxybutyryl-coa dehydrogenase activity
(s)-3-hydroxybutanoyl-coa:nadp+ oxidoreductase activity
l(+)-3-hydroxybutyryl-coa dehydrogenase activity
MainRecentTopDoctors