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L-serine
dehydratase/L-
threonine
deaminase
branched-chain
alpha-keto
dehydrogenase
complex
Propionyl-CoA
carboxylase
Methylmalonyl-
CoA epimerase,
mitochondrial
Methylmalonyl-
CoA mutase,
mitochondrial
Succinyl-CoA
2-Ketobutyric acid
L-Threonine
L-Threonine
2-Ketobutyric acid
NH
3
CoA
NAD
Propionyl-CoA
NADH
HCO
3
-
ATP
S-Methylmalonyl-CoA
ADP
H
+
R-Methylmalonyl-CoA
Thiamine
pyrophosphate
FAD
Thiamine
pyrophosphate
Biotin
Adenosylcobalamin
Citric Acid
Cycle
Homocysteine
Degradation
Methionine
Metabolism
Pyridoxal
5'-phosphate
Extracellular space
Intracellular Space
Mitochondria
Inner mitochondrial membrane
Outer mitochondrial membrane
Mitochondria
SDS
BCKDHA
PCCA
PCCB
MCEE
MUT
Succinyl-CoA
2-Ketobutyric
acid
L-Threonine
L-Threonine
2-Ketobutyric
acid
Ammonia
Coenzyme A
NAD
Propionyl-CoA
NADH
Hydrogen
carbonate
Adenosine
triphosphate
S-
Methylmalonyl-
CoA
Adenosine
diphosphate
Hydrogen
R-
Methylmalonyl-
CoA
Citric Acid
Cycle
Homocysteine
Degradation
Methionine
Metabolism
SDS
BCKDHA
PCCA
PCCB
MCEE
MUT
Suc-CoA
2KBA
Thr
Thr
2KBA
NH
3
CoA
NAD
PropCoA
NADH
HCO
3
-
ATP
S-MmCoA
ADP
H
+
R-MmCoA
ThiamPP
FAD
ThiamPP
Biotin
Adnscbn
PW000005
PW000027
PW000056