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
-
inborn
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3-methylcrotonyl-coa
-
isovalerylglycine
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medium-chain
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lethargy
-
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
-
3-hydroxyisovaleric
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octanoyl-coa
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sweaty
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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
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results (Natural Substrates Products
Natural Substrates Products on EC 1.3.8.4 - isovaleryl-CoA dehydrogenase
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NATURAL SUBSTRATE 
NATURAL 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
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 + 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
-
-
-
?
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
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
isovaleryl-CoA + acceptor
3-methylcrotonyl-CoA + reduced acceptor
-
-
-
?
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 + 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 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
-
-
-
?
isovaleryl-CoA + electron-transfer flavoprotein
3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
-
-
-
?
isovaleryl-CoA + electron-transfer flavoprotein
3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
-
-
-
?
additional information
?
-
-
the presence of a putative enzyme supports functional leucine catabolism in plant mitochondria
-
-
?
additional information
?
-
-
deficiency of the enzyme in humans is responsible for isovaleric acidemia
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-
?
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
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-
?
additional information
?
-
-
a specific deficiency of enzyme activity is observed in cultured skin fibroblasts from patients with isovaleric acidemia
-
-
?
additional information
?
-
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the enzyme catalyzes the third step of the leucine oxidative metabolism
-
-
?
additional information
?
-
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the enzyme catalyzes the third step of the leucine oxidative metabolism
-
-
?
additional information
?
-
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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
?
-
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the enzyme catalyzes the third step of the leucine oxidative metabolism
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-
?
additional information
?
-
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physiological implications of enzyme mutations and deficiency
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-
?
additional information
?
-
-
isovaleric acidemia is a rare recessive autosomal disorder, caused by isovaleryl-CoA dehydrogenase (IVD) deficiency
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-
?
additional information
?
-
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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
?
-
-
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
-
-
?
