1.1.1.178: 3-hydroxy-2-methylbutyryl-CoA dehydrogenase

This is an abbreviated version, for detailed information about 3-hydroxy-2-methylbutyryl-CoA dehydrogenase, go to the full flat file.

Reaction

(2S,3S)-3-hydroxy-2-methylbutanoyl-CoA
+
NAD+
=
2-methylacetoacetyl-CoA
+
NADH
+
H+

Synonyms

17beta-HSD10, 2-methyl-3-hydroxy-butyryl CoA dehydrogenase, 2-methyl-3-hydroxybutyryl coenzyme A dehydrogenase, 2-methyl-3-hydroxybutyryl-CoA dehydrogenase, 3-hydroxyacyl-CoA dehydrogenase type II, dehydrogenase, 2-methyl-3-hydroxybutyryl coenzyme A, HSD10, MHBD, short-chain L-3-hydroxy-2-methylacyl-CoA dehydrogenase

ECTree

     1 Oxidoreductases
         1.1 Acting on the CH-OH group of donors
             1.1.1 With NAD+ or NADP+ as acceptor
                1.1.1.178 3-hydroxy-2-methylbutyryl-CoA dehydrogenase

Engineering

Engineering on EC 1.1.1.178 - 3-hydroxy-2-methylbutyryl-CoA dehydrogenase

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ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
D86G
28% residual activity. Mutation causes severe disruption of mitochondrial morphology
L122V
-
nucleotide substitution C364G, inborn mutation involved in X-linked MHBD deficiency
N274S
-
nucleotide substitution A740G, inborn mutation involved in lethal X-linked MHBD deficiency
Q165H
complete loss of activity, no binding of cofactor NAD+ or NADH
additional information