EC Number |
Natural Substrates |
---|
1.3.8.4 | isovaleryl-CoA + 2,6-dichlorophenolindophenol |
high substrate specificity |
1.3.8.4 | isovaleryl-CoA + acceptor |
- |
1.3.8.4 | isovaleryl-CoA + acceptor |
electron acceptor: electron-transfer flavoprotein serves as a natural electron acceptor for the enzyme with isovaleryl-CoA as substrate |
1.3.8.4 | isovaleryl-CoA + electron transfer protein |
- |
1.3.8.4 | isovaleryl-CoA + electron-transfer flavoprotein |
- |
1.3.8.4 | isovaleryl-CoA + FAD |
- |
1.3.8.4 | more |
deficiency of the enzyme in humans is responsible for isovaleric acidemia |
1.3.8.4 | more |
Glu-244 is the catalytic base responsible for abstracting the alpha-proton of substrate |
1.3.8.4 | more |
E254 of the enzyme is in close proximity to the bound FAD, E254 is the active site catalytic residue |
1.3.8.4 | more |
the location of the catalytic residue together with a glycine at position 374 is important for conferring branched-chain substrate specificity to the enzyme |