1.3.8.5: short-chain 2-methylacyl-CoA dehydrogenase
This is an abbreviated version!
For detailed information about short-chain 2-methylacyl-CoA dehydrogenase, go to the full flat file.
Word Map on EC 1.3.8.5
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1.3.8.5
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acylcarnitine
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acadsb
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isobutyryl-coa
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dehydrogenation
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isovaleryl-coa
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acidemia
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isovaleric
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inborn
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hmong
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l-isoleucine
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ascarid
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isovalerylcarnitine
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acylglycine
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wisconsin
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submitochondrial
- 1.3.8.5
- acylcarnitine
-
acadsb
- isobutyryl-coa
-
dehydrogenation
- isovaleryl-coa
- acidemia
-
isovaleric
-
inborn
-
hmong
- l-isoleucine
-
ascarid
- isovalerylcarnitine
- acylglycine
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wisconsin
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submitochondrial
Reaction
Synonyms
2-methyl branched chain acyl-CoA dehydrogenase, 2-Methyl branched-chain acyl-CoA dehydrogenase, 2-Methyl branched-chain enoyl CoA reductase, 2-Methyl branched-chain enoyl-CoA reductase, 2-methylbutyryl-CoA dehydrogenase, 2MBCD, branched chain acyl-CoA dehydrogenase, EC 1.3.99.12, ECR, Methyl branched-chain enoyl-CoA reductase, Reductase, methyl branched-chain enoyl coenzyme A, SBCAD, SBCADD, short/branched chain acyl-CoA dehydrogenase
ECTree
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Substrates Products
Substrates Products on EC 1.3.8.5 - short-chain 2-methylacyl-CoA dehydrogenase
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REACTION DIAGRAM
(R)-2-methylbutanoyl-CoA + electron-transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
(S)-2-methylbutanoyl-CoA + electron-transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
2-methylbutanoyl-CoA + electron transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
2-methylbutanoyl-CoA + electron-transfer flavoprotein
(E)-2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
-
-
?
2-methylpentanoyl-CoA + electron-transfer flavoprotein
2-methyl-2-pentenoyl-CoA + reduced electron-transfer flavoprotein + H+
98% of the activity with 2-methylbutanoyl-CoA
-
?
butyryl-CoA + acceptor
2-butenoyl-CoA + reduced acceptor
-
5% of activity with 2-methylbutyryl-CoA
-
-
?
butyryl-CoA + electron-transfer flavoprotein
? + reduced electron-transfer flavoprotein + H+
13.6% of the activity with 2-methylbutanoyl-CoA
-
-
?
butyryl-CoA + electron-transfer flavoprotein
but-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
-
-
-
-
?
hexanoyl-CoA + electron-transfer flavoprotein
hex-2-enoyl-CoA + reduced electron-transfer flavoprotein
isobutyryl-CoA + electron-transfer flavoprotein
2-methylacryloyl-CoA + reduced electron-transfer flavoprotein + H+
octanoyl-CoA + electron-transfer flavoprotein
? + reduced electron-transfer flavoprotein + H+
12.8% of the activity with 2-methylbutanoyl-CoA
-
-
?
octanoyl-CoA + electron-transfer flavoprotein
oct-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
-
-
-
-
?
pentanoyl-CoA + electron-transfer flavoprotein
? + reduced electron-transfer flavoprotein + H+
14.4% of the activity with 2-methylbutanoyl-CoA
-
-
?
valeryl-CoA + electron-transfer flavoprotein
pent-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
-
-
-
-
?
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
-
-
i.e. ethylacrylyl-CoA
-
?
(R)-2-methylbutanoyl-CoA + electron-transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
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involved in the (S)-pathway of L-isoleucine oxidation, and also in the (R)-pathway of L-alloisoleucine oxidation due to interconvertion of the 3 isomers
-
-
?
2-ethylprop-2-enoyl-CoA + reduced acceptor
-
-
-
?
(R)-2-methylbutyryl-CoA + acceptor
2-ethylprop-2-enoyl-CoA + reduced acceptor
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22% of activity with (S)-2-methylbutyryl-CoA
-
?
2-methylbut-2-enoyl-CoA + reduced acceptor
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acceptors: electron transfer flavoprotein, phenazine methosulfate, meldola's blue
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r
(S)-2-methylbutanoyl-CoA + acceptor
2-methylbut-2-enoyl-CoA + reduced acceptor
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acceptors: electron transfer flavoprotein, phenazine methosulfate, meldola's blue
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r
(S)-2-methylbutanoyl-CoA + acceptor
2-methylbut-2-enoyl-CoA + reduced acceptor
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acceptors: electron transfer flavoprotein, phenazine methosulfate, meldola's blue
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r
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
-
-
-
-
?
(S)-2-methylbutanoyl-CoA + electron-transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
-
-
i.e. tiglyl-CoA
-
?
(S)-2-methylbutanoyl-CoA + electron-transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
-
involved in the (S)-pathway of L-isoleucine oxidation, and also in the (R)-pathway of L-alloisoleucine oxidation due to interconvertion of the 3 isomers
-
-
?
(S)-2-methylbutanoyl-CoA + electron-transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
-
-
-
-
?
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
-
-
-
r
2-methylbutanoyl-CoA + electron transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
-
-
-
r
2-methylbutanoyl-CoA + electron transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
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enzyme catalyzes the third step in the oxidative metabolism of isoleucin and valine
-
r
2-methylbutanoyl-CoA + electron transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
-
-
-
-
?
hex-2-enoyl-CoA + reduced electron-transfer flavoprotein
-
41% of activity with (S)-2-methylbutanoyl-CoA
-
-
?
hexanoyl-CoA + electron-transfer flavoprotein
hex-2-enoyl-CoA + reduced electron-transfer flavoprotein
-
8% of activity with (S)-2-methylbutanoyl-CoA, wild-type enzyme
-
-
?
hexanoyl-CoA + electron-transfer flavoprotein
hex-2-enoyl-CoA + reduced electron-transfer flavoprotein
-
-
-
-
?
methacrylyl-CoA + reduced acceptor
-
-
-
?
isobutyryl-CoA + acceptor
methacrylyl-CoA + reduced acceptor
-
-
-
?
isobutyryl-CoA + acceptor
methacrylyl-CoA + reduced acceptor
-
-
-
?
2-methylacryloyl-CoA + reduced electron-transfer flavoprotein + H+
-
6% of activity with (S)-2-methylbutanoyl-CoA
-
-
?
isobutyryl-CoA + electron-transfer flavoprotein
2-methylacryloyl-CoA + reduced electron-transfer flavoprotein + H+
-
37% of activity with (S)-2-methylbutanoyl-CoA, wild-type enzyme
-
-
?
?
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no reaction with: propionyl-CoA, isobutanoyl-CoA, isopentanoyl-CoA, palmitoyl-CoA
-
-
?
additional information
?
-
-
no reaction with: propionyl-CoA, isobutanoyl-CoA, isopentanoyl-CoA, palmitoyl-CoA
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-
?
additional information
?
-
-
the enzyme plays a pivotal role in the reversal of beta-oxidation operating in anaerobic mitochondria of the parasitic nematode Ascaris suum
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-
?
additional information
?
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the enzyme plays a pivotal role in the reversal of beta-oxidation operating in anaerobic mitochondria of the parasitic nematode Ascaris suum
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-
?
additional information
?
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the enzyme plays a pivotal role in the reversal of beta-oxidation operating in anaerobic mitochondria of the parasitic nematode Ascaris suum
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-
?
additional information
?
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the electron-transport chain of the organism and the enzyme 2-methyl-branched-chain-enoyl-CoA reductase are responsible for the NADH-dependent reduction of the branched-chain enoyl-CoAs observed in mitochondria
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-
?
additional information
?
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the electron-transport chain of the organism and the enzyme 2-methyl-branched-chain-enoyl-CoA reductase are responsible for the NADH-dependent reduction of the branched-chain enoyl-CoAs observed in mitochondria
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-
?
additional information
?
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expression of the enzyme is tissue-specific and developmentally regulated
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-
?
additional information
?
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enzyme deficiency causes 2-ethylhydracrylic aciduria with excretion f 2-methylbutyrylglycine
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-
?
additional information
?
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substrate specificity of wild-type and mutants, overview
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-
?