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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
chain
Acetyl-CoA
Succinyl-CoA
Propionic acid
Hydroxypropionic
acid
Propionyl-CoA
β-Alanine
2-Ketobutyric acid
Malonyl-CoA semialdehyde
3-Hydroxypropionyl-CoA
S-Methylmalonyl-CoA
R-Methylmalonyl-CoA
L-Valine
(S)-
Methylmalonic
acid
semialdehyde
Accumulation
Methylmalonic
acid
Malonyl-CoA
CO
2
ATP
Hydrogen
carbonate
P
i
ADP
Acetoacetyl-CoA
CoA
Malonic
semialdehyde
CoA
NAD
CO
2
NADH
Oxoglutaric
acid
L-Glutamic acid
Acrylyl-CoA
H
2
O
H
2
O
CoA
AMP
PP
i
Propinol adenylate
ATP
Pantetheine
ATP
Pantetheine
AMP
PP
i
AMP
AMP
H
2
O
NAD
CoA
NADH
Hydrogen
carbonate
Hydrogen
carbonate
ATP
ADP
H
+
CoA
NAD
NADH
2-Hydroxybutyric acid
NAD
NADH
H
+
Adenosylcobalamin
Biotin
Manganese
Pyridoxal
5'-phosphate
FAD
Biotin
FAD
Thiamine
pyrophosphate
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
Mitochondria
Mitochondrial Matrix
Intracellular Space
Extracellular Space