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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 alpha,
mitochondrial
2-
oxoisovalerate
dehydrogenase
subunit beta,
mitochondrial
2-
oxoisovalerate
dehydrogenase
subunit alpha,
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
Dihydrolipoyllysine-
residue
acetyltransferase
component of
pyruvate
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
Dihydrolipoyllysine-
residue
acetyltransferase
component of
pyruvate
dehydrogenase
complex,
mitochondrial
2-
oxoisovalerate
dehydrogenase
2-
oxoisovalerate
dehydrogenase
Dihydrolipoyllysine-
residue
acetyltransferase
component of
pyruvate
dehydrogenase
complex,
mitochondrial
Short-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Short/branched
chain specific
acyl-CoA
dehydrogenase,
mitochondrial
Short-chain
specific
acyl-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
Methylmalonyl
CoA mutase
Aldehyde
oxidase 1
Aldehyde
dehydrogenase,
mitochondrial
Methylglutaconyl-
CoA hydratase,
mitochondrial
Hydroxymethylglutaryl-
CoA synthase,
mitochondrial
Acetyl-CoA
acetyltransferase,
mitochondrial
Succinyl-
CoA:3- ketoacid
coenzyme A
transferase 1,
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
+
3-Hydroxy-3-methylglutaryl-CoA
Acetoacetic
acid
2-Methyl-1-hydroxypropyl-ThPP
Thiamine
pyrophosphate
S-(2-Methylpropionyl)-
dihydrolipoamide-E
Lipoamide
2-Methyl-1-hydroxypropyl-ThPP
S-(2-Methylbutanoyl)-
dihydrolipoamide
Thiamine pyrophosphate
L-Valine
Oxoglutaric
acid
α-
Ketoisovaleric
acid
L-Glutamic acid
L-Leucine
Oxoglutaric
acid
Ketoleucine
L-Glutamic acid
L-Isoleucine
Oxoglutaric
acid
3-Methyl-2-
oxovaleric acid
L-Glutamic acid
Thiamine pyrophosphate
CO
2
CoA
Accumulation
Isovaleryl-CoA
3-Methylcrotonyl-CoA
3-Hydroxyisovaleryl-CoA
H
2
O
ATP
Hydrogen
carbonate
3-Methylglutaconyl-CoA
ADP
Dihydrolipoamide
NAD
NADH
H
+
CoA
Isobutyryl-CoA
CO
2
CO
2
CoA
2-Methylbutyryl-CoA
Methacrylyl-CoA
H
2
O
(S)-3-Hydroxyisobutyryl-CoA
H
2
O
(S)-3-
Hydroxyisobutyric
acid
CoA
NAD
(S)-
Methylmalonic
acid
semialdehyde
NADH
H
+
Oxoglutaric
acid
L-Glutamic acid
Tiglyl-CoA
H
2
O
2-Methyl-3-hydroxybutyryl-CoA
NAD
2-Methylacetoacetyl-CoA
NADH
CoA
H
2
O
NAD
CoA
NADH
Hydrogen
carbonate
ATP
Hydrogen
carbonate
S-Methylmalonyl-CoA
ADP
P
i
R-Methylmalonyl-CoA
H
2
O
O
2
Methylmalonic
acid
Hydrogen
peroxide
NAD
H
2
O
NADH
H
2
O
Acetoacetyl-CoA
H
2
O
CoA
CoA
Succinyl-CoA
Succinic acid
Thiamine
pyrophosphate
FAD
Thiamine
pyrophosphate
Pyridoxal
Pyridoxal
Pyridoxal
Pyridoxal
5'-phosphate
Lipoyl-GMP
FAD
FAD
Biotin
FAD
Lipoyl-GMP