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
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1.3.8.4
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acidemia
-
acyl-coa
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inborn
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3-methylcrotonyl-coa
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isovalerylglycine
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medium-chain
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lethargy
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flavoenzyme
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isobutyryl-coa
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acylcarnitine
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isovalerylcarnitine
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flavoprotein:ubiquinone
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glutaryl-coa
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2-methylbutyryl-coa
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3-hydroxyisovaleric
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octanoyl-coa
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sweaty
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diagnostics
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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
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Specific Activity
Specific Activity on EC 1.3.8.4 - isovaleryl-CoA dehydrogenase
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0.000039
-
mitochondria from isovaleric acidemia cells, 13% relative activity to normal cells, specific activity determined by tritium release assay
0.000163
-
mitochondria from isovaleric acidemia cells, 12% relative activity to normal cells, specific activity determined by dye reduction assay
0.0003
-
recombinant mutant L95V/A99V/L103V/L370M/G374A, substrate 2-methylbutanoyl-CoA
0.00031
-
mitochondria from normal cells, specific activity determined by tritium release assay
0.00034
fibroblast cell line harboring the mutation corresponding to the A282V mutant
0.00044
-
sonicated mitochondria, acceptor added: electron-transferring flavoprotein
0.00131
-
mitochondria from normal cells, specific activity determined by dye reduction assay
0.004
cell free extract of Escherichia coli cells expressing the enzyme mutant R363C
0.0041
0.0062
-
purified enzyme in the absence of electron transfer agents, reaction terminated with HCl or with KMnO4/HCl
0.01
-
propionyl-CoA as substrate and phenazine methosulfate as acceptor or hexanoyl-CoA as substrate and 0.0056 mM electron transfer flavoprotein as acceptor
0.0136
-
purified enzyme with phenazine methosulfate and dichlorophenol indophenol, reaction terminated with HCl
0.0207
-
purified enzyme with electron-transferring flavoprotein and 0.062 mM dichlorophenol indophenol, reaction terminated with HCl, concentration of isovaleryl-CoA: 0.05 mM, specific activity determined by tritium release assay
0.03
0.032
cell free extract of Escherichia coli cells expressing the enzyme mutant R382L
0.043
0.0472
-
purified enzyme with phenazine methosulfate and dichlorophenol indophenol, reaction terminated with KMnO4/HCl
0.052
cell free extract of Escherichia coli cells expressing the enzyme mutant V342A
0.0572
-
purified enzyme with electron-transferring flavoprotein and 0.062 mM dichlorophenol indophenol, reaction terminated with KMnO4/HCl, concentration of isovaleryl-CoA: 0.05 mM, specific activity determined by tritium release assay
0.086
0.09
-
valeryl-CoA as substrate and 0.0056 mM electron transfer flavoprotein as acceptor
0.17
-
S-2-methylbutyryl-CoA as substrate and phenazine methosulfate as acceptor
0.172
-
purified enzyme, acceptor added: electron-transferring flavoprotein and 0.062 mM dichlorophenol indophenol, concentration of isovaleryl-CoA: 0.02 mM, specific activity determined by dye reduction assay
0.44
cell free extract of Escherichia coli cells expressing the enzyme, wild type
0.53
-
isovaleryl-CoA as substrate and 0.0056 mM electron transfer flavoprotein as acceptor
0.6
of purified recombinant V342A mutant, electron acceptor: electron-transferring flavoprotein
0.72
-
isovaleryl-CoA as substrate and 0.012 mM electron transfer flavoprotein as acceptor
0.832
1.24
of purified recombinant V342A mutant, electron acceptor: dichlorophenol indophenol + FAD
1.27
of purified recombinant V342A mutant, electron acceptor: dichlorophenol indophenol
1.4
of purified recombinant R382L mutant, electron acceptor: electron-transferring flavoprotein
10.3
of purified recombinant wild-type enzyme, electron acceptor: dichlorophenol indophenol + FAD
11.7
of purified recombinant wild-type enzyme, electron acceptor: electron-transferring flavoprotein
2.04
of purified recombinant A282V mutant, electron acceptor: dichlorophenol indophenol
2.2
of purified recombinant A282V mutant, electron acceptor: electron-transferring flavoprotein
2.33
of purified recombinant A282V mutant, electron acceptor: dichlorophenol indophenol + FAD
2.68
4.88
of purified recombinant R382L mutant, electron acceptor: dichlorophenol indophenol
6.34
of purified recombinant R382L mutant, electron acceptor: dichlorophenol indophenol + FAD
8.2
of purified recombinant wild-type enzyme, electron acceptor: dichlorophenol indophenol
-
butyryl-CoA as substrate and 0.0056 mM electron transfer flavoprotein as acceptor
-
purified enzyme, acceptor added: electron-transferring flavoprotein and 0.062 mM dichlorophenol indophenol, reaction terminated with KMnO4/HCl, concentration of isovaleryl-CoA: 0.02 mM, specific activity determined by tritium release assay
cell free extract of Escherichia coli cells expressing the enzyme mutant A282V