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Literature summary extracted from
- Characterization of molecular defects in isovaleryl-CoA dehydrogenase in patients with isovaleric acidemia (1998), Biochemistry, 37, 10325-10335.
Cloned(Commentary)
| EC Number | Cloned (Comment) | Organism |
|---|---|---|
| 1.3.8.4 | expression of normal and mutant enzymes in Escherichia coli | Homo sapiens |
Protein Variants
| EC Number | Protein Variants | Comment | Organism |
|---|---|---|---|
| 1.3.8.4 | A282V | enzyme activity detected, 19% relative activity to wild-type | Homo sapiens |
| 1.3.8.4 | B40N | has no detectable enzymatic activity | Homo sapiens |
| 1.3.8.4 | C328R | has no detectable enzymatic activity | Homo sapiens |
| 1.3.8.4 | L13P | has no detectable enzymatic activity | Homo sapiens |
| 1.3.8.4 | R21P | has no detectable enzymatic activity | Homo sapiens |
| 1.3.8.4 | R363C | enzyme activity detected, 1% relative activity to wild-type | Homo sapiens |
| 1.3.8.4 | R382L | enzyme activity detected, 7% relative activity to wild-type | Homo sapiens |
| 1.3.8.4 | V342A | enzyme activity detected, 12% relative activity to wild-type | Homo sapiens |
KM Value [mM]
| EC Number | KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|---|
| 1.3.8.4 | 0.0028 | - |
isovaleryl-CoA | purified enzyme V342A | Homo sapiens |  |
| 1.3.8.4 | 0.0028 | - |
isovaleryl-CoA | purified V342A mutant, electron acceptor: electron-transferring flavoprotein | Homo sapiens | |
| 1.3.8.4 | 0.0031 | - |
isovaleryl-CoA | purified wild-type enzyme, electron acceptor: electron-transferring flavoprotein | Homo sapiens | |
| 1.3.8.4 | 0.0045 | - |
isovaleryl-CoA | purified R382L mutant, electron acceptor: dichlorophenol indophenol | Homo sapiens | |
| 1.3.8.4 | 0.0069 | - |
isovaleryl-CoA | purified R382L mutant, electron acceptor: electron-transferring flavoprotein | Homo sapiens | |
| 1.3.8.4 | 0.024 | - |
isovaleryl-CoA | purified V342A mutant, electron acceptor: dichlorophenol indophenol | Homo sapiens | |
| 1.3.8.4 | 0.027 | - |
isovaleryl-CoA | purified A282V mutant, electron acceptor: electron-transferring flavoprotein | Homo sapiens | |
| 1.3.8.4 | 0.0296 | - |
isovaleryl-CoA | purified wild-type enzyme, electron acceptor: dichlorophenol indophenol | Homo sapiens | |
| 1.3.8.4 | 0.41 | - |
isovaleryl-CoA | purified A282V mutant, electron acceptor: dichlorophenol indophenol | Homo sapiens | |
Localization
| EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|---|
| 1.3.8.4 | mitochondrion | - |
Homo sapiens | 5739 | - |
Natural Substrates/ Products (Substrates)
| EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 1.3.8.4 | isovaleryl-CoA + acceptor | Homo sapiens | - |
3-methylcrotonyl-CoA + reduced acceptor | - |
? | |
| 1.3.8.4 | additional information | Homo sapiens | deficiency of the enzyme in humans is responsible for isovaleric acidemia | ? | - |
? | |
| 1.3.8.4 | additional information | Homo sapiens | the enzyme catalyzes the third step of the leucine oxidative metabolism | ? | - |
? | |
Organism
| EC Number | Organism | UniProt | Comment | Textmining |
|---|---|---|---|---|
| 1.3.8.4 | Homo sapiens | P26440 | - |
- |
Purification (Commentary)
| EC Number | Purification (Comment) | Organism |
|---|---|---|
| 1.3.8.4 | by loading the supernatant from an induced culture onto an XK16/20 Fast Q fast flow column, overnight dialysis in potassium phosphate buffer, and hydroxyapatite column chromatography | Homo sapiens |
Source Tissue
| EC Number | Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|---|
| 1.3.8.4 | fibroblast | - |
Homo sapiens | - |
Specific Activity [micromol/min/mg]
| EC Number | Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
|---|---|---|---|---|
| 1.3.8.4 | 0.00034 | - |
fibroblast cell line harboring the mutation corresponding to the A282V mutant | Homo sapiens |
| 1.3.8.4 | 0.004 | - |
cell free extract of Escherichia coli cells expressing the enzyme mutant R363C | Homo sapiens |
| 1.3.8.4 | 0.0041 | - |
normal cell lines | Homo sapiens |
| 1.3.8.4 | 0.032 | - |
cell free extract of Escherichia coli cells expressing the enzyme mutant R382L | Homo sapiens |
| 1.3.8.4 | 0.052 | - |
cell free extract of Escherichia coli cells expressing the enzyme mutant V342A | Homo sapiens |
| 1.3.8.4 | 0.086 | - |
cell free extract of Escherichia coli cells expressing the enzyme mutant A282V | Homo sapiens |
| 1.3.8.4 | 0.44 | - |
cell free extract of Escherichia coli cells expressing the enzyme, wild type | Homo sapiens |
| 1.3.8.4 | 0.6 | - |
of purified recombinant V342A mutant, electron acceptor: electron-transferring flavoprotein | Homo sapiens |
| 1.3.8.4 | 1.24 | - |
of purified recombinant V342A mutant, electron acceptor: dichlorophenol indophenol + FAD | Homo sapiens |
| 1.3.8.4 | 1.27 | - |
of purified recombinant V342A mutant, electron acceptor: dichlorophenol indophenol | Homo sapiens |
| 1.3.8.4 | 1.4 | - |
of purified recombinant R382L mutant, electron acceptor: electron-transferring flavoprotein | Homo sapiens |
| 1.3.8.4 | 2.04 | - |
of purified recombinant A282V mutant, electron acceptor: dichlorophenol indophenol | Homo sapiens |
| 1.3.8.4 | 2.2 | - |
of purified recombinant A282V mutant, electron acceptor: electron-transferring flavoprotein | Homo sapiens |
| 1.3.8.4 | 2.33 | - |
of purified recombinant A282V mutant, electron acceptor: dichlorophenol indophenol + FAD | Homo sapiens |
| 1.3.8.4 | 4.88 | - |
of purified recombinant R382L mutant, electron acceptor: dichlorophenol indophenol | Homo sapiens |
| 1.3.8.4 | 6.34 | - |
of purified recombinant R382L mutant, electron acceptor: dichlorophenol indophenol + FAD | Homo sapiens |
| 1.3.8.4 | 8.2 | - |
of purified recombinant wild-type enzyme, electron acceptor: dichlorophenol indophenol | Homo sapiens |
| 1.3.8.4 | 10.3 | - |
of purified recombinant wild-type enzyme, electron acceptor: dichlorophenol indophenol + FAD | Homo sapiens |
| 1.3.8.4 | 11.7 | - |
of purified recombinant wild-type enzyme, electron acceptor: electron-transferring flavoprotein | Homo sapiens |
Substrates and Products (Substrate)
| EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 1.3.8.4 | isovaleryl-CoA + acceptor | - |
Homo sapiens | 3-methylcrotonyl-CoA + reduced acceptor | - |
? | |
| 1.3.8.4 | isovaleryl-CoA + acceptor | electron acceptor: electron-transferring flavoprotein, dichlorophenol indophenol or dichlorophenol indophenol + FAD | Homo sapiens | 3-methylcrotonyl-CoA + reduced acceptor | - |
? | |
| 1.3.8.4 | additional information | deficiency of the enzyme in humans is responsible for isovaleric acidemia | Homo sapiens | ? | - |
? | |
| 1.3.8.4 | additional information | the enzyme catalyzes the third step of the leucine oxidative metabolism | Homo sapiens | ? | - |
? | |
Turnover Number [1/s]
| EC Number | Turnover Number Minimum [1/s] | Turnover Number Maximum [1/s] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|---|
| 1.3.8.4 | 0.283 | - |
isovaleryl-CoA | purified V342A mutant, electron acceptor: dichlorophenol indophenol | Homo sapiens | |
| 1.3.8.4 | 0.733 | - |
isovaleryl-CoA | purified R382L mutant, electron acceptor: dichlorophenol indophenol | Homo sapiens | |
| 1.3.8.4 | 0.817 | - |
isovaleryl-CoA | purified V342A mutant, electron acceptor: electron-transferring flavoprotein | Homo sapiens | |
| 1.3.8.4 | 2 | - |
isovaleryl-CoA | purified R382L mutant, electron acceptor: electron-transferring flavoprotein | Homo sapiens | |
| 1.3.8.4 | 2.2 | - |
isovaleryl-CoA | purified A282V mutant, electron acceptor: electron-transferring flavoprotein | Homo sapiens | |
| 1.3.8.4 | 2.82 | - |
isovaleryl-CoA | purified A282V mutant, electron acceptor: dichlorophenol indophenol | Homo sapiens | |
| 1.3.8.4 | 8.8 | - |
isovaleryl-CoA | purified wild-type enzyme, electron acceptor: electron-transferring flavoprotein | Homo sapiens | |
| 1.3.8.4 | 10 | - |
isovaleryl-CoA | purified wild-type enzyme, electron acceptor: dichlorophenol indophenol | Homo sapiens | |
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