EC Number | Cloned (Comment) | Organism |
---|---|---|
1.3.8.4 | cDNA modified at its 5'-end coding region to enhance expression in Escherichia coli | Homo sapiens |
EC Number | Protein Variants | Comment | Organism |
---|---|---|---|
1.3.8.4 | E254D | has residual activity for isovaleryl-CoA, below 0.1% | Homo sapiens |
1.3.8.4 | E254G | has no detectable enzymatic activity | Homo sapiens |
1.3.8.4 | E254G/A375E | exhibits catalytic activity toward isovaleryl-CoA | Homo sapiens |
1.3.8.4 | E254Q | has no detectable enzymatic activity | Homo sapiens |
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 | E254 of the enzyme is in close proximity to the bound FAD, E254 is the active site catalytic residue | ? | - |
? | |
1.3.8.4 | additional information | Homo sapiens | the enzyme catalyzes the third step of the leucine oxidative metabolism | ? | - |
? |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
1.3.8.4 | Homo sapiens | - |
- |
- |
EC Number | Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|---|
1.3.8.4 | 0.08 | - |
substrate: octanoyl-CoA | Homo sapiens |
1.3.8.4 | 1.19 | - |
substrate: hexanoyl-CoA | Homo sapiens |
1.3.8.4 | 1.71 | - |
substrate: butyryl-CoA | Homo sapiens |
1.3.8.4 | 3.71 | - |
substrate: valeryl-CoA | Homo sapiens |
1.3.8.4 | 8.1 | - |
substrate: isovaleryl-CoA | Homo sapiens |
EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
1.3.8.4 | butyryl-CoA + acceptor | 21% relative specific activity to isovaleryl-CoA | Homo sapiens | but-2-enoyl-CoA + reduced acceptor | - |
? | |
1.3.8.4 | hexanoyl-CoA + acceptor | 15% relative specific activity to isovaleryl-CoA | Homo sapiens | hex-2-enoyl-CoA + reduced acceptor | - |
? | |
1.3.8.4 | isovaleryl-CoA + acceptor | - |
Homo sapiens | 3-methylcrotonyl-CoA + reduced acceptor | - |
? | |
1.3.8.4 | isovaleryl-CoA + acceptor | the catalytic activity of the recombinant wild-type enzyme is the highest in the presence of isovaleryl-CoA | Homo sapiens | 3-methylcrotonyl-CoA + reduced acceptor | - |
? | |
1.3.8.4 | isovaleryl-CoA + acceptor | acceptors: electron-transfer flavoprotein, phenazine methosulfate | 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 | E254 of the enzyme is in close proximity to the bound FAD, E254 is the active site catalytic residue | Homo sapiens | ? | - |
? | |
1.3.8.4 | additional information | the enzyme catalyzes the third step of the leucine oxidative metabolism | Homo sapiens | ? | - |
? | |
1.3.8.4 | n-valeryl-CoA + acceptor | 46% relative specific activity to isovaleryl-CoA | Homo sapiens | pent-2-enoyl-CoA + reduced acceptor | - |
? | |
1.3.8.4 | octanoyl-CoA + acceptor | 1% relative specific activity to isovaleryl-CoA | Homo sapiens | oct-2-enoyl-CoA + reduced acceptor | - |
? |
EC Number | Cofactor | Comment | Organism | Structure |
---|---|---|---|---|
1.3.8.4 | FAD | - |
Homo sapiens |