1.3.8.4: isovaleryl-CoA dehydrogenase
This is an abbreviated version!
For detailed information about isovaleryl-CoA dehydrogenase, go to the full flat file.
Word Map on EC 1.3.8.4
-
1.3.8.4
-
acidemia
-
acyl-coa
-
inborn
-
3-methylcrotonyl-coa
-
isovalerylglycine
-
medium-chain
-
lethargy
-
flavoenzyme
-
isobutyryl-coa
-
acylcarnitine
-
isovalerylcarnitine
-
flavoprotein:ubiquinone
-
glutaryl-coa
-
2-methylbutyryl-coa
-
3-hydroxyisovaleric
-
octanoyl-coa
-
sweaty
-
diagnostics
-
medicine
- 1.3.8.4
-
acidemia
- acyl-coa
-
inborn
- 3-methylcrotonyl-coa
-
isovalerylglycine
-
medium-chain
-
lethargy
-
flavoenzyme
- isobutyryl-coa
- acylcarnitine
- isovalerylcarnitine
-
flavoprotein:ubiquinone
- glutaryl-coa
- 2-methylbutyryl-coa
-
3-hydroxyisovaleric
- octanoyl-coa
-
sweaty
- diagnostics
- medicine
Reaction
Synonyms
acyl-CoA dehydrogenase, EC 1.3.99.10, i3VD, iso(3)valeryl-CoA dehydrogenase, isovaleric-CoA dehydrogenase, isovaleroyl-coenzyme A dehydrogenase, isovaleryl-coenzyme A dehydrogenase, IVD, IVDH, LiuA, Pden_3633, SBCAD, short/branched chain acyl-CoA dehydrogenase
ECTree
1.3.8.4 isovaleryl-CoA dehydrogenaseAdvanced search results
results (
results (Substrates Products
Substrates Products on EC 1.3.8.4 - isovaleryl-CoA dehydrogenase
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SUBSTRATE 
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(S)-2-methylbutyryl-CoA + electron-transfer flavoprotein
2-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
-
-
-
?
2-methyl-hexanoyl-CoA + acceptor
2-methylhex-2-enoyl-CoA + reduced acceptor
-
4% relative activity to isovaleryl-CoA
-
?
2-methylbutanoyl-CoA + acceptor
2-methylbut-2-enoyl-CoA + reduced acceptor
-
low activity with the wild-type and mutant L95V/A99V/L103V/L370M/G374A
-
-
?
butyryl-CoA + 2,6-dichlorophenolindophenol
?
-
high substrate specificity
-
-
?
butyryl-CoA + electron-transfer flavoprotein
crotonyl-CoA + reduced electron-transfer flavoprotein
-
-
-
?
hexanoyl-CoA + electron-transfer flavoprotein
hex-2-enoyl-CoA + reduced electron-transfer flavoprotein
-
-
-
?
isovaleryl-CoA + 2,6-dichlorophenolindophenol
?
-
high substrate specificity
-
-
?
isovaleryl-CoA + electron transfer protein
3-methylcrotonoyl-CoA + reduced electron transfer protein
isovaleryl-CoA + electron transfer protein
3-methylcrotonyl-CoA + reduced electron transfer protein
isovaleryl-CoA + electron-transfer flavoprotein
3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
isovaleryl-CoA + phenazine methosulfate
3-methylcrotonyl-CoA + reduced phenazine methosulfate
2-methylpalmitoyl-CoA + acceptor
2-methylhexadec-2-enoyl-CoA + reduced acceptor
-
7% relative activity to isovaleryl-CoA
-
?
2-methylpalmitoyl-CoA + acceptor
2-methylhexadec-2-enoyl-CoA + reduced acceptor
-
minor activity
-
?
2-methylpalmitoyl-CoA + acceptor
2-methylhexadec-2-enoyl-CoA + reduced acceptor
-
minor activity
-
?
2-methylpalmitoyl-CoA + acceptor
2-methylhexadec-2-enoyl-CoA + reduced acceptor
-
minor activity
-
?
butyryl-CoA + acceptor
but-2-enoyl-CoA + reduced acceptor
-
electron acceptor: electron-transferring flavoprotein
-
?
butyryl-CoA + acceptor
but-2-enoyl-CoA + reduced acceptor
-
acceptor: phenazine methosulfate
-
?
butyryl-CoA + acceptor
but-2-enoyl-CoA + reduced acceptor
-
21% relative specific activity to isovaleryl-CoA
-
?
butyryl-CoA + acceptor
but-2-enoyl-CoA + reduced acceptor
-
3.3% relative activity to isovaleryl-CoA
-
?
butyryl-CoA + acceptor
but-2-enoyl-CoA + reduced acceptor
low activity
-
-
?
hexanoyl-CoA + acceptor
hex-2-enoyl-CoA + reduced acceptor
-
weak activity
-
?
hexanoyl-CoA + acceptor
hex-2-enoyl-CoA + reduced acceptor
-
electron acceptor: electron-transferring flavoprotein
-
?
hexanoyl-CoA + acceptor
hex-2-enoyl-CoA + reduced acceptor
-
acceptor: phenazine methosulfate
-
?
hexanoyl-CoA + acceptor
hex-2-enoyl-CoA + reduced acceptor
-
15% relative specific activity to isovaleryl-CoA
-
?
hexanoyl-CoA + acceptor
hex-2-enoyl-CoA + reduced acceptor
low activity
-
-
?
isobutyryl-CoA + acceptor
2-methylpropanoyl-CoA + reduced acceptor
-
20% relative activity to isovaleryl-CoA
-
?
isobutyryl-CoA + acceptor
2-methylpropanoyl-CoA + reduced acceptor
-
the activity with isobutyryl-CoA implies an additional role of the enzyme in the catabolism of valine
-
?
isobutyryl-CoA + acceptor
2-methylpropanoyl-CoA + reduced acceptor
-
the activity with isobutyryl-CoA implies an additional role of the enzyme in the catabolism of valine
-
?
isobutyryl-CoA + acceptor
2-methylpropanoyl-CoA + reduced acceptor
-
the activity with isobutyryl-CoA implies an additional role of the enzyme in the catabolism of valine
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
isovaleryl-CoA is the preferred substrate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
isovaleryl-CoA is the preferred substrate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
electron acceptor: ferricenium hexafluorophosphate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
isovaleryl-CoA is the preferred substrate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
electron acceptor: electron-transferring flavoprotein
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
enzyme activity observed in the absence of electron transfer agents suggests that rapid tritium exchange occurs between the enzyme-bound substrate and the incubation medium
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
the catalytic activity of the recombinant wild-type enzyme is the highest in the presence of isovaleryl-CoA
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
electron acceptor: electron-transferring flavoprotein
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
electron acceptor: electron-transferring flavoprotein, dichlorophenol indophenol or dichlorophenol indophenol + FAD
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
isovaleryl-CoA is the preferred substrate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
isovaleryl-CoA is the preferred substrate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
isovaleryl-CoA is the preferred substrate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
isovaleryl-CoA is the preferred substrate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
acceptors: electron-transfer flavoprotein, phenazine methosulfate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
electron acceptor: electron-transfer flavoprotein serves as a natural electron acceptor for the enzyme with isovaleryl-CoA as substrate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
electron acceptor: electron-transfer flavoprotein serves as a natural electron acceptor for the enzyme with isovaleryl-CoA as substrate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
isovaleryl-CoA is the preferred substrate
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
electron acceptor: 2,6-dichloroindophenol, intermediate electron carrier: phenazine methosulfate
-
?
isovaleryl-CoA + electron transfer protein
3-methylcrotonoyl-CoA + reduced electron transfer protein
-
-
-
-
?
isovaleryl-CoA + electron transfer protein
3-methylcrotonoyl-CoA + reduced electron transfer protein
acceptor porcine electron transfer protein
-
-
?
isovaleryl-CoA + electron transfer protein
3-methylcrotonyl-CoA + reduced electron transfer protein
-
-
-
-
?
isovaleryl-CoA + electron transfer protein
3-methylcrotonyl-CoA + reduced electron transfer protein
-
-
-
?
isovaleryl-CoA + electron transfer protein
3-methylcrotonyl-CoA + reduced electron transfer protein
best substrate
-
-
?
isovaleryl-CoA + electron-transfer flavoprotein
3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
-
-
-
?
isovaleryl-CoA + electron-transfer flavoprotein
3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
-
-
-
?
isovaleryl-CoA + electron-transfer flavoprotein
3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
-
-
-
-
?
isovaleryl-CoA + electron-transfer flavoprotein
3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
-
reaction via an alpha,beta-dehydrogenation step
-
-
?
isovaleryl-CoA + electron-transfer flavoprotein
3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
-
-
-
-
r
isovaleryl-CoA + electron-transfer flavoprotein
3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
-
-
-
?
isovaleryl-CoA + electron-transfer flavoprotein
3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
-
-
-
?
isovaleryl-CoA + electron-transfer flavoprotein
3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
-
-
-
?
isovaleryl-CoA + FAD
3-methylcrotonyl-CoA + FADH2
with ferrocenium hexafluorophosphate as second electron acceptor
-
-
?
isovaleryl-CoA + phenazine methosulfate
3-methylcrotonyl-CoA + reduced phenazine methosulfate
-
-
-
-
?
isovaleryl-CoA + phenazine methosulfate
3-methylcrotonyl-CoA + reduced phenazine methosulfate
-
-
-
-
?
n-valeryl-CoA + acceptor
pent-2-enoyl-CoA + reduced acceptor
-
electron acceptor: electron-transferring flavoprotein
-
?
n-valeryl-CoA + acceptor
pent-2-enoyl-CoA + reduced acceptor
-
46% relative specific activity to isovaleryl-CoA
-
?
n-valeryl-CoA + acceptor
pent-2-enoyl-CoA + reduced acceptor
-
-
-
?
n-valeryl-CoA + acceptor
pent-2-enoyl-CoA + reduced acceptor
-
-
-
?
n-valeryl-CoA + acceptor
pent-2-enoyl-CoA + reduced acceptor
-
32% relative specific activity to isovaleryl-CoA
-
?
octanoyl-CoA + acceptor
oct-2-enoyl-CoA + reduced acceptor
-
7% relative activity to isovaleryl-CoA
-
?
octanoyl-CoA + acceptor
oct-2-enoyl-CoA + reduced acceptor
-
1% relative specific activity to isovaleryl-CoA
-
?
octanoyl-CoA + acceptor
oct-2-enoyl-CoA + reduced acceptor
very low activity
-
-
?
valeryl-CoA + acceptor
pent-2-enoyl-CoA + reduced acceptor
low activity
-
-
?
additional information
?
-
-
the presence of a putative enzyme supports functional leucine catabolism in plant mitochondria
-
-
?
additional information
?
-
activity measurement using an acyl-CoA substrate and 2,6-dichloroindophenol as an electron acceptor, with phenazinemethosulfate as an intermediate electron carrier
-
-
?
additional information
?
-
-
activity measurement using an acyl-CoA substrate and 2,6-dichloroindophenol as an electron acceptor, with phenazinemethosulfate as an intermediate electron carrier
-
-
?
additional information
?
-
activity measurement using an acyl-CoA substrate and 2,6-dichloroindophenol as an electron acceptor, with phenazinemethosulfate as an intermediate electron carrier
-
-
?
additional information
?
-
activity measurement using an acyl-CoA substrate and 2,6-dichloroindophenol as an electron acceptor, with phenazinemethosulfate as an intermediate electron carrier
-
-
?
additional information
?
-
-
substrate specificity of wild-type and mutant enzymes
-
-
?
additional information
?
-
-
deficiency of the enzyme in humans is responsible for isovaleric acidemia
-
-
?
additional information
?
-
deficiency of the enzyme in humans is responsible for isovaleric acidemia
-
-
?
additional information
?
-
-
deficiency of the enzyme in humans is responsible for isovaleric acidemia
-
-
?
additional information
?
-
-
E254 of the enzyme is in close proximity to the bound FAD, E254 is the active site catalytic residue
-
-
?
additional information
?
-
-
E254 of the enzyme is in close proximity to the bound FAD, E254 is the active site catalytic residue
-
-
?
additional information
?
-
-
the location of the catalytic residue together with a glycine at position 374 is important for conferring branched-chain substrate specificity to the enzyme
-
-
?
additional information
?
-
-
R387 has an important role in anchoring the substrate
-
-
?
additional information
?
-
-
the type III enzyme mutation responsible for isovaleric acidemia leads to a shift in reading frame and the subsequent incorporation of eight abnormally placed amino acids with premature termination of translation
-
-
?
additional information
?
-
-
a specific deficiency of enzyme activity is observed in cultured skin fibroblasts from patients with isovaleric acidemia
-
-
?
additional information
?
-
-
a specific deficiency of enzyme activity is observed in cultured skin fibroblasts from patients with isovaleric acidemia
-
-
?
additional information
?
-
-
the enzyme catalyzes the third step of the leucine oxidative metabolism
-
-
?
additional information
?
-
-
the enzyme catalyzes the third step of the leucine oxidative metabolism
-
-
?
additional information
?
-
-
the enzyme catalyzes the third step of the leucine oxidative metabolism
-
-
?
additional information
?
-
the enzyme catalyzes the third step of the leucine oxidative metabolism
-
-
?
additional information
?
-
-
the enzyme catalyzes the third step of the leucine oxidative metabolism
-
-
?
additional information
?
-
-
physiological implications of enzyme mutations and deficiency
-
-
?
additional information
?
-
-
isovaleric acidemia is a rare recessive autosomal disorder, caused by isovaleryl-CoA dehydrogenase (IVD) deficiency
-
-
?
additional information
?
-
-
Glu-244 is the catalytic base responsible for abstracting the alpha-proton of substrate
-
-
?
additional information
?
-
-
the enzyme is required in the leucine/isovalerate utilization, Liu, pathway for growth of Pseudomonas aeruginosa on acyclic terpene alcohols (citronellol) and on other methyl-branched compounds such as leucine or isovalerate, Liu proteins in metabolism of methyl-branched compounds, overview
-
-
?
additional information
?
-
-
no activity of LiuA with isobutyryl-CoA, 3-hydroxybutyryl-CoA, octanoyl-CoA, citronellyl-CoA or 5-methyl-hex-4-enoyl-CoA
-
-
?
additional information
?
-
-
the enzyme catalyzes the third step of the leucine oxidative metabolism
-
-
?
additional information
?
-
the enzyme catalyzes the dehydrogenation of monomethyl branched-chain fatty acid thioester derivatives
-
-
?
additional information
?
-
broader substrate specificity, overview. The enzyme is also active with octanoyl-CoA, decanoyl-CoA, and dodecanoyl-CoA. No activity with isobutyryl-CoA and myristoyl-CoA, with DCIPIP and phenazine methosulfate as second electron acceptors
-
-
?
