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(R)-2-methylbutanoyl-CoA + electron-transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+
(R)-2-methylbutyryl-CoA + acceptor
2-ethylprop-2-enoyl-CoA + reduced acceptor
(S)-2-methylbutanoyl-CoA + acceptor
2-methylbut-2-enoyl-CoA + reduced acceptor
(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 + acceptor
methacrylyl-CoA + reduced acceptor
isobutyryl-CoA + electron-transfer flavoprotein
2-methylacryloyl-CoA + reduced electron-transfer flavoprotein + H+
isovaleryl-CoA + phenazine methosulfate
?
-
-
-
-
r
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+
-
-
-
-
?
additional information
?
-
(R)-2-methylbutanoyl-CoA + electron-transfer flavoprotein
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+
-
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
-
-
?
(R)-2-methylbutyryl-CoA + acceptor
2-ethylprop-2-enoyl-CoA + reduced acceptor
-
-
-
?
(R)-2-methylbutyryl-CoA + acceptor
2-ethylprop-2-enoyl-CoA + reduced acceptor
-
22% of activity with (S)-2-methylbutyryl-CoA
-
?
(S)-2-methylbutanoyl-CoA + acceptor
2-methylbut-2-enoyl-CoA + reduced acceptor
-
acceptors: electron transfer flavoprotein, phenazine methosulfate, meldola's blue
-
r
(S)-2-methylbutanoyl-CoA + acceptor
2-methylbut-2-enoyl-CoA + reduced acceptor
-
acceptors: electron transfer flavoprotein, phenazine methosulfate, meldola's blue
-
r
(S)-2-methylbutanoyl-CoA + acceptor
2-methylbut-2-enoyl-CoA + reduced acceptor
-
acceptors: electron transfer flavoprotein, phenazine methosulfate, meldola's blue
-
r
(S)-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+
-
-
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-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
-
-
-
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
-
r
2-methylbutanoyl-CoA + electron transfer flavoprotein
2-methylbut-2-enoyl-CoA + reduced electron transfer flavoprotein
-
-
-
-
?
hexanoyl-CoA + 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
-
-
-
-
?
isobutyryl-CoA + acceptor
methacrylyl-CoA + reduced acceptor
-
-
-
?
isobutyryl-CoA + acceptor
methacrylyl-CoA + reduced acceptor
-
-
-
?
isobutyryl-CoA + acceptor
methacrylyl-CoA + reduced acceptor
-
-
-
?
isobutyryl-CoA + electron-transfer flavoprotein
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
-
-
?
additional information
?
-
no reaction with: propionyl-CoA, isobutanoyl-CoA, isopentanoyl-CoA, palmitoyl-CoA
-
-
?
additional information
?
-
-
no reaction with: propionyl-CoA, isobutanoyl-CoA, isopentanoyl-CoA, palmitoyl-CoA
-
-
?
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
?
-
-
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
?
-
-
expression of the enzyme is tissue-specific and developmentally regulated
-
-
?
additional information
?
-
-
enzyme deficiency causes 2-ethylhydracrylic aciduria with excretion f 2-methylbutyrylglycine
-
-
?
additional information
?
-
-
substrate specificity of wild-type and mutants, overview
-
-
?
additional information
?
-
-
no activity with isovaleryl-CoA
-
-
?
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(R)-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+
-
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
additional information
?
-
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
-
-
-
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
-
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
?
-
-
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
?
-
-
expression of the enzyme is tissue-specific and developmentally regulated
-
-
?
additional information
?
-
-
enzyme deficiency causes 2-ethylhydracrylic aciduria with excretion f 2-methylbutyrylglycine
-
-
?
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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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Komuniecki, R.; Fekete, S.; Thissen-Parra, J.
Purification and characterization of the 2-methyl branched-chain acyl-CoA dehydrogenase, an enzyme involved in NADH-dependent enoyl-CoA reduction in anaerobic mitochondria of the nematode, Ascaris suum
J. Biol. Chem.
260
4770-4777
1985
Ascaris suum (O16843), Ascaris suum
brenda
Mei, B.; Komuniecki, R.; Komunieki, P.R.
Localization of cytochrome oxidase and the 2-methyl branched-chain enoyl CoA reductase in muscle and hypodermis of Ascaris suum larvae and adults
J. Parasitol.
83
760-763
1997
Ascaris suum
brenda
Duran, E.; Komuniecki, R.W.; Komuniecki, P.R.; Wheelock, M.J.; Klingbeil, M.L.; Ma, Y.; Johnson, K.R.
Characterization of cDNA clones for the 2-methyl branched-chain enoyl CoA reductase: an enzyme involved in branched-chain fatty acid synthesis in anaerobic mitochondria of the nematode, Ascaris suum
J. Biol. Chem.
268
22391-22396
1993
Ascaris suum
brenda
Duran, E.; Walker, D.J.; Johnson, K.R.; Komuniecki, P.R.; Komuniecki, R.W.
Developmental and tissue-specific expression of 2-methyl branched-chain enoyl CoA reductase isoforms in the parasitic nematode, Ascaris suum
Mol. Biochem. Parasitol.
91
307-318
1998
Ascaris suum
brenda
Duran, E.
Characterization of enoly CoA reductase isoforms: enzymes involved in branched-chain fatty acids synthesis in anaerobic mitochondria of the parasitic nematode, Ascaris suum
Diss. Abstr. Int.
57
670
1997
Ascaris suum
-
brenda
Ikeda, Y.; Dabrowski, C.; Tanaka, K.
Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria. Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase
J. Biol. Chem.
258
1066-1076
1983
Rattus norvegicus
brenda
Ikeda, Y.; Tanaka, K.
2-Methyl branched-chain acyl-CoA dehydrogenase from rat liver
Methods Enzymol.
166
360-373
1988
Rattus norvegicus
brenda
Ikeda, Y.; Tanaka, K.
Purification and characterization of 2-methyl-branched chain acyl coenzyme a dehydrogenase, an enzyme involved in the isoleucine and valine metabolism, from rat liver mitochondria
J. Biol. Chem.
258
9477-9487
1983
Rattus norvegicus
brenda
Ikeda, Y.; Tanaka, K.
Selective inactivation of various acyl-CoA dehydrogenases by (methylenecyclopropyl)acetyl-CoA
Biochim. Biophys. Acta
1038
216-221
1990
Rattus norvegicus
brenda
Korman, S.H.; Andresen, B.S.; Zeharia, A.; Gutman, A.; Boneh, A.; Pitt, J.J.
2-Ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation
Clin. Chem.
51
610-617
2005
Homo sapiens
brenda
He, M.; Burghardt, T.P.; Vockley, J.
A novel approach to the characterization of substrate specificity in short/branched chain acyl-CoA dehydrogenase
J. Biol. Chem.
278
37974-37986
2003
Homo sapiens, Rattus norvegicus
brenda
Goetzman, E.S.; Mohsen, A.W.; Prasad, K.; Vockley, J.
Convergent evolution of a 2-methylbutyryl-CoA dehydrogenase from isovaleryl-CoA dehydrogenase in Solanum tuberosum
J. Biol. Chem.
280
4873-4879
2005
Solanum tuberosum
brenda