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

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The expected taxonomic range for this enzyme is: Eukaryota, Bacteria

EC NUMBER
COMMENTARY hide
1.1.1.178
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RECOMMENDED NAME
GeneOntology No.
3-hydroxy-2-methylbutyryl-CoA dehydrogenase
REACTION
REACTION DIAGRAM
COMMENTARY hide
ORGANISM
UNIPROT
LITERATURE
(2S,3S)-3-hydroxy-2-methylbutanoyl-CoA + NAD+ = 2-methylacetoacetyl-CoA + NADH + H+
show the reaction diagram
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
oxidation
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-
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redox reaction
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-
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reduction
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-
-
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PATHWAY
BRENDA Link
KEGG Link
MetaCyc Link
2-methylbutanoate biosynthesis
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Biosynthesis of antibiotics
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Biosynthesis of secondary metabolites
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L-isoleucine degradation I
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Metabolic pathways
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Valine, leucine and isoleucine degradation
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SYSTEMATIC NAME
IUBMB Comments
(2S,3S)-3-hydroxy-2-methylbutanoyl-CoA:NAD+ oxidoreductase
Also acts, more slowly, on (2S,3S)-2-hydroxy-3-methylpentanoyl-CoA.
CAS REGISTRY NUMBER
COMMENTARY hide
52227-66-4
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ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
bifunctional 17beta-hydroxysteroid dehydrogenase type 10/2-methyl-3-hydroxybutyryl-CoA dehydrogenase
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Manually annotated by BRENDA team
PpG2
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Manually annotated by BRENDA team
PpG2
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Manually annotated by BRENDA team
Sprague-Dawley rats
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Manually annotated by BRENDA team
bifunctional 17beta-hydroxysteroid dehydrogenase type 10/2-methyl-3-hydroxybutyryl-CoA dehydrogenase
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-
Manually annotated by BRENDA team
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
physiological function
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
2-hydroxy-3-methylpentanoyl-CoA + NAD+
3-methyl-2-oxopentanoyl-CoA + NADH
show the reaction diagram
2-methyl-3-hydroxybutyryl-CoA + NAD+
2-methylacetoacetyl-CoA + NADH
show the reaction diagram
2-methyl-3-hydroxybutyryl-CoA + NAD+
2-methylacetoacetyl-CoA + NADH + H+
show the reaction diagram
-
-
-
-
?
3-hydroxybutyryl-CoA + NAD+
acetoacetyl-CoA + NADH
show the reaction diagram
additional information
?
-
NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
2-methyl-3-hydroxybutyryl-CoA + NAD+
2-methylacetoacetyl-CoA + NADH
show the reaction diagram
additional information
?
-
COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.037
2-methyl-3-hydroxybutyryl-CoA
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0.5
3-hydroxybutyryl-CoA
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
16.2
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2-methyl-3-hydroxybutyryl-CoA
21.7
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3-hydroxybutyryl-CoA
additional information
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remaining enzyme activity in genetic deficient fibroblast is 0.02 nmol per min and mg protein
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
30
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assay at
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
PDB
SCOP
CATH
ORGANISM
UNIPROT
Mycobacterium avium (strain 104)
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
27000
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immunoblot analysis
66000
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gel filtration
130000
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gel filtration, sucrose density gradient centrifugation
SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
dimer
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2 * 28000, SDS-PAGE
Purification/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
6-step procedure with yield of only 6%
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ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
D86G
28% residual activity. Mutation causes severe disruption of mitochondrial morphology
L122V
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nucleotide substitution C364G, inborn mutation involved in X-linked MHBD deficiency
N274S
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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
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine
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