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Information on EC 1.3.8.5 - short-chain 2-methylacyl-CoA dehydrogenase
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EC Tree
1.3.8.5 short-chain 2-methylacyl-CoA dehydrogenaseIUBMB Comments
A flavoprotein (FAD). The mammalian enzyme catalyses an oxidative reaction as a step in the mitochondrial beta-oxidation of short-chain 2-methyl fatty acids and participates in isoleucine degradation. The enzyme from the parasitic helminth Ascaris suum catalyses a reductive reaction as part of a fermentation pathway, shuttling reducing power from the electron-transport chain to 2-methyl branched-chain enoyl CoA.
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Word Map
- 1.3.8.5
- acylcarnitine
-
acadsb
- isobutyryl-coa
-
dehydrogenation
- isovaleryl-coa
- acidemia
-
isovaleric
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inborn
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hmong
- l-isoleucine
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ascarid
- isovalerylcarnitine
- acylglycine
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wisconsin
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submitochondrial
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
Reaction Schemes
Synonyms
sbcad, sbcadd, short/branched chain acyl-coa dehydrogenase, 2-methylbutyryl-coa dehydrogenase, 2mbcd, 2-methyl branched-chain acyl-coa dehydrogenase, 2-methyl branched-chain enoyl-coa reductase, 2-methyl branched-chain enoyl coa reductase, 2-methyl branched chain acyl-coa dehydrogenase, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
2-methyl branched chain acyl-CoA dehydrogenase
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2-Methyl branched-chain acyl-CoA dehydrogenase
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2-Methyl branched-chain enoyl CoA reductase
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2-Methyl branched-chain enoyl-CoA reductase
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branched chain acyl-CoA dehydrogenase
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EC 1.3.99.12
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formerly
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ECR
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Methyl branched-chain enoyl-CoA reductase
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Reductase, methyl branched-chain enoyl coenzyme A
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SBCAD
short/branched chain acyl-CoA dehydrogenase
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
2-methylbutanoyl-CoA + electron-transfer flavoprotein = (E)-2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
2-methylbutanoyl-CoA + electron-transfer flavoprotein = (E)-2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
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2-methylbutanoyl-CoA + electron-transfer flavoprotein = (E)-2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
reaction mechanism determining substrate specificity
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2-methylbutanoyl-CoA + electron-transfer flavoprotein = (E)-2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
reaction mechanism determining substrate specificity
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2-methylbutanoyl-CoA + electron-transfer flavoprotein = (E)-2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
the isovaleryl-CoA dehydrogenase 1, i.e. IVD1, EC 1.3.99.10, from Solanum tuberosum shows maximal activity with 2-methylbutyryl-CoA, the binding pockets of 2-methylbutyryl-CoA and isovaleryl-CoA dehydrogenase are nearly identical, the enzyme therefore shall also be classified to EC 1.3.99.12, active site structure, residues Met361, Ala365, Val88, Val92, and Val96 are involved in catalysis
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
oxidation
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reduction
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PATHWAY SOURCE
PATHWAYS
BRENDA
KEGG
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SYSTEMATIC NAME
IUBMB Comments
short-chain 2-methylacyl-CoA:electron-transfer flavoprotein 2-oxidoreductase
A flavoprotein (FAD). The mammalian enzyme catalyses an oxidative reaction as a step in the mitochondrial beta-oxidation of short-chain 2-methyl fatty acids and participates in isoleucine degradation. The enzyme from the parasitic helminth Ascaris suum catalyses a reductive reaction as part of a fermentation pathway, shuttling reducing power from the electron-transport chain to 2-methyl branched-chain enoyl CoA.
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CAS REGISTRY NUMBER
COMMENTARY
122320-06-3
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SUBSTRATE
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+
(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+
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-
?
2-methylpentanoyl-CoA + electron-transfer flavoprotein
2-methyl-2-pentenoyl-CoA + reduced electron-transfer flavoprotein + H+
98% of the activity with 2-methylbutanoyl-CoA
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?
butyryl-CoA + acceptor
2-butenoyl-CoA + reduced acceptor
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5% of activity with 2-methylbutyryl-CoA
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?
butyryl-CoA + electron-transfer flavoprotein
? + reduced electron-transfer flavoprotein + H+
13.6% of the activity with 2-methylbutanoyl-CoA
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-
?
butyryl-CoA + electron-transfer flavoprotein
but-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
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-
-
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?
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
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-
?
octanoyl-CoA + electron-transfer flavoprotein
oct-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
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-
-
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?
pentanoyl-CoA + electron-transfer flavoprotein
? + reduced electron-transfer flavoprotein + H+
14.4% of the activity with 2-methylbutanoyl-CoA
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-
?
valeryl-CoA + electron-transfer flavoprotein
pent-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
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-
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?
(R)-2-methylbutanoyl-CoA + electron-transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
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i.e. ethylacrylyl-CoA
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?
(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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?
(R)-2-methylbutyryl-CoA + acceptor
2-ethylprop-2-enoyl-CoA + reduced acceptor
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?
(R)-2-methylbutyryl-CoA + acceptor
2-ethylprop-2-enoyl-CoA + reduced acceptor
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22% of activity with (S)-2-methylbutyryl-CoA
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?
(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
(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 + electron-transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
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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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i.e. tiglyl-CoA
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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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?
(S)-2-methylbutanoyl-CoA + electron-transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
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?
2-methylbutanoyl-CoA + electron transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
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r
2-methylbutanoyl-CoA + electron transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
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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
2-methylbutanoyl-CoA + electron transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
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?
hexanoyl-CoA + electron-transfer flavoprotein
hex-2-enoyl-CoA + reduced electron-transfer flavoprotein
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41% of activity with (S)-2-methylbutanoyl-CoA
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?
hexanoyl-CoA + electron-transfer flavoprotein
hex-2-enoyl-CoA + reduced electron-transfer flavoprotein
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8% of activity with (S)-2-methylbutanoyl-CoA, wild-type enzyme
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?
hexanoyl-CoA + electron-transfer flavoprotein
hex-2-enoyl-CoA + reduced electron-transfer flavoprotein
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?
isobutyryl-CoA + acceptor
methacrylyl-CoA + reduced acceptor
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?
isobutyryl-CoA + acceptor
methacrylyl-CoA + reduced acceptor
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?
isobutyryl-CoA + acceptor
methacrylyl-CoA + reduced acceptor
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?
isobutyryl-CoA + electron-transfer flavoprotein
2-methylacryloyl-CoA + reduced electron-transfer flavoprotein + H+
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6% of activity with (S)-2-methylbutanoyl-CoA
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?
isobutyryl-CoA + electron-transfer flavoprotein
2-methylacryloyl-CoA + reduced electron-transfer flavoprotein + H+
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37% of activity with (S)-2-methylbutanoyl-CoA, wild-type enzyme
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?
additional information
?
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no reaction with: propionyl-CoA, isobutanoyl-CoA, isopentanoyl-CoA, palmitoyl-CoA
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?
additional information
?
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no reaction with: propionyl-CoA, isobutanoyl-CoA, isopentanoyl-CoA, palmitoyl-CoA
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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 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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?
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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
-
-
?
(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
-
-
?
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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-
-
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
-
enzyme catalyzes the third step in the oxidative metabolism of isoleucin and valine
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r
additional information
?
-
-
the enzyme plays a pivotal role in the reversal of beta-oxidation operating in anaerobic mitochondria of the parasitic nematode Ascaris suum
-
-
?
additional information
?
-
-
the enzyme plays a pivotal role in the reversal of beta-oxidation operating in anaerobic mitochondria of the parasitic nematode Ascaris suum
-
-
?
additional information
?
-
-
the enzyme plays a pivotal role in the reversal of beta-oxidation operating in anaerobic mitochondria of the parasitic nematode Ascaris suum
-
-
?
additional information
?
-
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
-
-
?
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
-
-
?
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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-
?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
(methylenecyclopropyl)acetyl-CoA
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0.0025 mM, slow inhibition, half-time approx. 30 min
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
acyl-coa dehydrogenase deficiency
2-methylbutyryl-CoA dehydrogenase deficiency associated with autism and mental retardation: a case report.
acyl-coa dehydrogenase deficiency
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
acyl-coa dehydrogenase deficiency
Biochemical, Clinical, and Genetic Characteristics of Short/Branched Chain Acyl-CoA Dehydrogenase Deficiency in Chinese Patients by Newborn Screening.
acyl-coa dehydrogenase deficiency
Prevalence and mutation analysis of short/branched chain acyl-CoA dehydrogenase deficiency (SBCADD) detected on newborn screening in Wisconsin.
Intellectual Disability
2-methylbutyryl-CoA dehydrogenase deficiency associated with autism and mental retardation: a case report.
Maple Syrup Urine Disease
Advances and challenges in the treatment of branched-chain amino/keto acid metabolic defects.
medium-chain acyl-coa dehydrogenase deficiency
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
methylcrotonoyl-coa carboxylase deficiency
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
Multiple Acyl Coenzyme A Dehydrogenase Deficiency
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
Propionic Acidemia
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
Propionic Acidemia
Advances and challenges in the treatment of branched-chain amino/keto acid metabolic defects.
Seizures
Short/branched-chain acyl-CoA dehydrogenase deficiency due to an IVS3+3A>G mutation that causes exon skipping.
short-chain 2-methylacyl-coa dehydrogenase deficiency
2-methylbutyryl-CoA dehydrogenase deficiency associated with autism and mental retardation: a case report.
short-chain 2-methylacyl-coa dehydrogenase deficiency
2-methylbutyryl-CoA dehydrogenase deficiency in Hmong infants identified by expanded newborn screen.
short-chain 2-methylacyl-coa dehydrogenase deficiency
2-Methylbutyrylglycine induces lipid oxidative damage and decreases the antioxidant defenses in rat brain.
short-chain 2-methylacyl-coa dehydrogenase deficiency
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
short-chain 2-methylacyl-coa dehydrogenase deficiency
Advances and challenges in the treatment of branched-chain amino/keto acid metabolic defects.
short-chain 2-methylacyl-coa dehydrogenase deficiency
Biochemical, Clinical, and Genetic Characteristics of Short/Branched Chain Acyl-CoA Dehydrogenase Deficiency in Chinese Patients by Newborn Screening.
short-chain 2-methylacyl-coa dehydrogenase deficiency
Characterization of new ACADSB gene sequence mutations and clinical implications in patients with 2-methylbutyrylglycinuria identified by newborn screening.
short-chain 2-methylacyl-coa dehydrogenase deficiency
Clinical, biochemical, and molecular spectrum of short/branched-chain acyl-CoA dehydrogenase deficiency: two new cases and review of literature.
short-chain 2-methylacyl-coa dehydrogenase deficiency
Inborn errors of isoleucine degradation: a review.
short-chain 2-methylacyl-coa dehydrogenase deficiency
Prevalence and mutation analysis of short/branched chain acyl-CoA dehydrogenase deficiency (SBCADD) detected on newborn screening in Wisconsin.
short-chain 2-methylacyl-coa dehydrogenase deficiency
Prospective diagnosis of 2-methylbutyryl-CoA dehydrogenase deficiency in the Hmong population by newborn screening using tandem mass spectrometry.
short-chain 2-methylacyl-coa dehydrogenase deficiency
UPLC-MS/MS analysis of C5-acylcarnitines in dried blood spots.
short-chain acyl-coa dehydrogenase deficiency
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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TURNOVER NUMBER [1/s]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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Ki VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
2.345
2.476
additional information
additional information
-
activity of wild-type and mutant enzymes with different substrates, overview
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
32
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
additional information
-
not detected in: early, aerobic larval stages or adult testis, ovary, or intstine
brenda
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
Show AA Sequence and Transmembrane Helices (132 entries)
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Please use the AA Sequence and Transmembrane Helices Search for a specific query.