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2-
oxoisovalerate
dehydrogenase
subunit beta,
mitochondrial
2-
oxoisovalerate
dehydrogenase
subunit alpha,
mitochondrial
Dihydrolipoyl
dehydrogenase,
mitochondrial
Hydroxymethylglutaryl-
CoA lyase,
mitochondrial
2-
oxoisovalerate
dehydrogenase
subunit beta,
mitochondrial
2-
oxoisovalerate
dehydrogenase
subunit beta,
mitochondrial
Branched-
chain-amino-
acid
aminotransferase,
cytosolic
Branched-
chain-amino-
acid
aminotransferase,
cytosolic
Branched-
chain-amino-
acid
aminotransferase,
cytosolic
2-
oxoisovalerate
dehydrogenase
Lipoamide
acyltransferase
component of
branched-chain
alpha-keto acid
dehydrogenase
complex,
mitochondrial
Isovaleryl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Methylcrotonoyl-
CoA carboxylase
subunit alpha,
mitochondrial
Methylcrotonoyl-
CoA carboxylase
beta chain,
mitochondrial
Dihydrolipoyl
dehydrogenase,
mitochondrial
Lipoamide
acyltransferase
component of
branched-chain
alpha-keto acid
dehydrogenase
complex,
mitochondrial
2-
oxoisovalerate
dehydrogenase
2-
oxoisovalerate
dehydrogenase
Lipoamide
acyltransferase
component of
branched-chain
alpha-keto acid
dehydrogenase
complex,
mitochondrial
Short-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Short/branched
chain specific
acyl-CoA
dehydrogenase,
mitochondrial
Isobutyryl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
3-
hydroxyisobutyryl-
CoA hydrolase,
mitochondrial
3-
hydroxyisobutyrate
dehydrogenase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
4-aminobutyrate
aminotransferase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Short-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Short/branched
chain specific
acyl-CoA
dehydrogenase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
3- hydroxyacyl-
CoA
dehydrogenase
type-2
3-ketoacyl-CoA
thiolase,
mitochondrial
Methylmalonate-
semialdehyde
dehydrogenase
[acylating],
mitochondrial
Propionyl-CoA
carboxylase
alpha chain,
mitochondrial
Propionyl-CoA
carboxylase
beta chain,
mitochondrial
Methylmalonyl-
CoA epimerase,
mitochondrial
Methylmalonyl-
CoA mutase,
mitochondrial
Aldehyde
oxidase
Aldehyde
dehydrogenase,
mitochondrial
Methylglutaconyl-
CoA hydratase,
mitochondrial
Hydroxymethylglutaryl-
CoA synthase,
mitochondrial
Acetyl-CoA
acetyltransferase,
mitochondrial
Succinyl-
CoA:3- ketoacid
coenzyme A
transferase 1,
mitochondrial
2-
oxoisovalerate
dehydrogenase
subunit alpha,
mitochondrial
2-
oxoisovalerate
dehydrogenase
subunit alpha,
mitochondrial
(S)-beta-Aminoisobutyric acid
Acetyl-CoA
Propionyl-CoA
Acetyl-CoA
Succinyl-CoA
3-Methyl-1-hydroxybutyl-ThPP
Lipoamide
S-(3-Methylbutanoyl)-dihydrolipoamide-E
Dihydrolipoamide
NAD
NADH
H
+