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
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acadsb
- isobutyryl-coa
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dehydrogenation
- isovaleryl-coa
- acidemia
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isovaleric
-
inborn
-
hmong
- l-isoleucine
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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
1.3.8.5 short-chain 2-methylacyl-CoA dehydrogenaseAdvanced search results
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results (Natural Substrates Products
Natural Substrates Products on EC 1.3.8.5 - short-chain 2-methylacyl-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
(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
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-
?
(S)-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
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-
?
2-methylbutanoyl-CoA + electron transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
2-methylbutanoyl-CoA + electron transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
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-
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r
2-methylbutanoyl-CoA + electron transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
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-
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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
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r
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 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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?
