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Mitochondrial
carnitine/acylcarnitine
carrier protein
Mitochondrial
carnitine/acylcarnitine
carrier protein
Carnitine O-
palmitoyltransferase
1, liver
isoform
Long-chain-
fatty-acid--
CoA ligase 1
Carnitine O-
palmitoyltransferase
2,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
Hydroxyacyl-
coenzyme A
dehydrogenase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
Hydroxyacyl-
coenzyme A
dehydrogenase,
mitochondrial
3-ketoacyl-CoA
thiolase,
mitochondrial
3-ketoacyl-CoA
thiolase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
Hydroxyacyl-
coenzyme A
dehydrogenase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
3-ketoacyl-CoA
thiolase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
Long-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Long-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Long-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Stearic acid
Acetyl-CoA
Acetyl-CoA
Acetyl-CoA
Lauroyl-CoA
L-Carnitine
L-Carnitine
Stearoylcarnitine
Stearoylcarnitine
Stearoyl-CoA
CoA
CoA
ATP
PP
i
AMP
Stearoyl-CoA
CoA
(2E)-Octadecenoyl-CoA
H
2
O
(S)-Hydroxyoctadecanoyl-CoA
H
2
O
NAD
3-oxooctadecanoyl-CoA
NADH
H
+
NAD
NADH
H
+
CoA
Palmitoyl-CoA
3-Hydroxyhexadecanoyl-CoA
(2E)-Hexadecenoyl-CoA
H
2
O
H
2
O
NAD
3-oxohexadecanoyl-CoA
NADH
H
+
Tetradecanoyl-CoA
CoA
CoA
CoA
NAD
NADH
H
+
3-Hydroxytetradecanoyl-CoA
(2E)-Tetradecenoyl-CoA
H
2
O
H
2
O
NAD
3-oxotetradecanoyl-CoA
NADH
H
+
NAD
NADH
H
+
CoA
CoA
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
Fatty Acid
Biosynthesis
Fatty Acid
Elongation in
Mitochondria
Citric Acid
Cycle
Citric Acid
Cycle
Citric Acid
Cycle
Mitochondrial
Beta-Oxidation
of Medium Chain
Saturated Fatty
Acids
Magnesium
FAD
FAD
FAD
Extracellular Space
Intracellular Space
Mitochondrial Outer Membrane
Mitochondrial Inner Membrane
Mitochondrial Matrix
Mitochondria
Mitochondria
SLC25A20
SLC25A20
CPT1A
ACSL1
CPT2
ECHS1
HADHA
HADHB
HADH
HADHA