Methylmalonyl-CoA
epimerase,
mitochondrial
Methylmalonyl-CoA
mutase,
mitochondrial
Malonyl-CoA
decarboxylase,
mitochondrial
Acetyl-CoA
carboxylase 1
Acetyl-CoA
acetyltransferase,
mitochondrial
Methylmalonate-
semialdehyde
dehydrogenase
[acylating],
mitochondrial
4-aminobutyrate
aminotransferase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
3-
hydroxyisobutyryl-
CoA hydrolase,
mitochondrial
Medium-chain
specific acyl-CoA
dehydrogenase,
mitochondrial
Acetyl-coenzyme A
synthetase
2-like,
mitochondrial
Acyl-CoA
synthetase
short-chain
family member 3,
mitochondrial
Acyl-CoA
synthetase
short-chain
family member 3,
mitochondrial
Acetyl-coenzyme A
synthetase
2-like,
mitochondrial
Methylmalonate-
semialdehyde
dehydrogenase
[acylating],
mitochondrial
Propionyl-CoA
carboxylase alpha
chain,
mitochondrial
Propionyl-CoA
carboxylase beta
chain,
mitochondrial
branched-chain
alpha-keto
dehydrogenase
complex
L-lactate
dehydrogenase
A-like 6B
Acetyl-CoA
Succinyl-CoA
Propionic acid
Hydroxypropionic
acid
Propionyl-CoA
β-Alanine
2-Ketobutyric
acid
Citric acid
Malonyl-CoA
semialdehyde
3-
Hydroxypropionyl-
CoA
S- Methylmalonyl-
CoA
R- Methylmalonyl-
CoA
L-Valine
(S)-Methylmalonic
acid semialdehyde
Methylmalonic
acid
Malonyl-CoA
CO2
ATP
HCO3-
Pi
ADP
Acetoacetyl-CoA
CoA
Malonic
semialdehyde
CoA
NAD
CO2
NADH
Oxoglutaric acid
L-Glutamic acid
Acrylyl-CoA
H2O
H2O
CoA
AMP
PPi
Propinol
adenylate
ATP
Pantetheine
ATP
Pantetheine
AMP
PPi
AMP
AMP
H2O
NAD
CoA
NADH
HCO3-
HCO3-
ATP
ADP
H+
CoA
NAD
NADH
2-Hydroxybutyric
acid
NAD
NADH
H+
Adenosylcobalamin
Pyridoxal
5'-phosphate
FAD
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
Pyruvate
Metabolism
Citric Acid
Cycle
Valine,
Leucine, and
Isoleucine
Degradation
Pantothenate
and CoA
Biosynthesis
Cysteine and
Methionine
Metabolism
Glycine, Serine,
and Threonine
Metabolism
Tricarboxylate
Transport System
Biotin
Manganese
Biotin
FAD
Thiamine
pyrophosphate
Mitochondria
Mitochondrial Matrix
Extracellular Space