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Mitochondria
BCKDHB
BCKDHA
DLD
HMGCL
BCKDHB
BCKDHB
BCAT1
BCAT1
BCAT1
BCKDHA
BCKDHB
DBT
IVD
ACADM
ECHS1
MCCC1
MCCC2
DLD
DBT
BCKDHA
BCKDHB
BCKDHA
BCKDHB
DBT
ACADS
ACADSB
ACAD8
ACADM
ECHS1
HIBCH
HIBADH
ECHS1
ABAT
ACADM
ACADS
ACADSB
ECHS1
HSD17B10
ACAA2
ALDH6A1
PCCA
PCCB
MCEE
MUT
AOX1
ALDH2
AUH
HMGCS2
ACAT1
OXCT1
BCKDHA
BCKDHA
(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
Hydrogen Ion
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
Carbon dioxide
Coenzyme A
Isovaleryl-CoA
3-
Methylcrotonyl-
CoA
3-
Hydroxyisovaleryl-
CoA
Water
Adenosine
triphosphate
Hydrogen
carbonate
3-
Methylglutaconyl-
CoA
Adenosine
diphosphate
Dihydrolipoamide
NAD
NADH
Hydrogen Ion
Coenzyme A
Isobutyryl-CoA
Carbon dioxide
Carbon dioxide
Coenzyme A
2-
Methylbutyryl-
CoA
Methacrylyl-CoA
Water
(S)-3-
Hydroxyisobutyryl-
CoA
Water
(S)-3-
Hydroxyisobutyric
acid
Coenzyme A
NAD
(S)-
Methylmalonic
acid
semialdehyde
NADH
Hydrogen Ion
Oxoglutaric
acid
L-Glutamic acid
Tiglyl-CoA
Water
2-Methyl-3-
hydroxybutyryl-
CoA
NAD
2-
Methylacetoacetyl-
CoA
NADH
Coenzyme A
Water
NAD
Coenzyme A
NADH
Hydrogen
carbonate
Adenosine
triphosphate
Hydrogen
carbonate
S-
Methylmalonyl-
CoA
Adenosine
diphosphate
Phosphate
R-
Methylmalonyl-
CoA
Water
Oxygen
Methylmalonic
acid
Hydrogen
peroxide
NAD
Water
NADH
Water
Acetoacetyl-CoA
Water
Coenzyme A
Coenzyme A
Succinyl-CoA
Succinic acid
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