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+
3-Hydroxy-3-
methylglutaryl-
CoA
Acetoacetic acid
2-Methyl-1-
hydroxypropyl-
ThPP
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
CO2
CoA
Isovaleryl-CoA
3-
Methylcrotonyl-
CoA
3-
Hydroxyisovaleryl-
CoA
H2O
ATP
HCO3-
3-
Methylglutaconyl-
CoA
ADP
Dihydrolipoamide
NAD
NADH
H+
CoA
Isobutyryl-CoA
CO2
CO2
CoA
2- Methylbutyryl-
CoA
Methacrylyl-CoA
H2O
(S)-3-
Hydroxyisobutyryl-
CoA
H2O
(S)-3-
Hydroxyisobutyric
acid
CoA
NAD
(S)-Methylmalonic
acid semialdehyde
NADH
H+
Oxoglutaric acid
L-Glutamic acid
Tiglyl-CoA
H2O
2-Methyl-3-
hydroxybutyryl-
CoA
NAD
2-
Methylacetoacetyl-
CoA
NADH
CoA
H2O
NAD
CoA
NADH
HCO3-
ATP
HCO3-
S- Methylmalonyl-
CoA
ADP
Pi
R- Methylmalonyl-
CoA
H2O
O2
Methylmalonic
acid
H2O2
NAD
H2O
NADH
H2O
Acetoacetyl-CoA
H2O
CoA
CoA
Succinyl-CoA
Succinic acid
Pyridoxal
Pyridoxal
FAD
FAD
FAD
FAD
FAD
Biotin
Adenosylcobalamin
FAD
Molybdopterin
2Fe-2S
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
Pyrimidine
Metabolism
Citric Acid
Cycle
Propanoate
Metabolism
Citric Acid
Cycle
Citric Acid
Cycle
Thiamine
pyrophosphate
Thiamine
pyrophosphate
FAD
Thiamine
pyrophosphate
Pyridoxal
Pyridoxal
5'-phosphate
Lipoyl-GMP
FAD
FAD
Biotin
FAD
Lipoyl-GMP
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
Lipoyl-GMP
FAD
FAD
Pyridoxal
5'-phosphate
Mitochondria
Cytosol