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Mitochondrial
basic amino
acids
transporter
Acyl-coenzyme A
synthetase
ACSM1,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
HADHA protein
Trifunctional
enzyme subunit
beta,
mitochondrial
Hydroxyacyl-CoA
dehydrogenase
HADHA protein
Trifunctional
enzyme subunit
beta,
mitochondrial
3-ketoacyl-CoA
thiolase,
mitochondrial
HADHA protein
Trifunctional
enzyme subunit
beta,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
HADHA protein
Trifunctional
enzyme subunit
beta,
mitochondrial
Hydroxyacyl-CoA
dehydrogenase
HADHA protein
Trifunctional
enzyme subunit
beta,
mitochondrial
3-ketoacyl-CoA
thiolase,
mitochondrial
HADHA protein
Trifunctional
enzyme subunit
beta,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
HADHA protein
Trifunctional
enzyme subunit
beta,
mitochondrial
Hydroxyacyl-CoA
dehydrogenase
HADHA protein
Trifunctional
enzyme subunit
beta,
mitochondrial
3-ketoacyl-CoA
thiolase,
mitochondrial
HADHA protein
Trifunctional
enzyme subunit
beta,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Dodecanoic acid
Lauroyl-CoA
Acetyl-CoA
Acetyl-CoA
Acetyl-CoA
Hexanoyl-CoA
Dodecanoic acid
ATP
CoA
AMP
PP
i
3-Hydroxydodecanoyl-CoA
(2E)-Dodecenoyl-CoA
H
2
O
H
2
O
NAD
3-Oxododecanoyl-CoA
NADH
H
+
NAD
NADH
H
+
Decanoyl-CoA
CoA
CoA
3-Hydroxydecanoyl-CoA
(2E)-Decenoyl-CoA
H
2
O
H
2
O
NAD
3-Oxodecanoyl-CoA
H
+
NADH
NAD
H
+
NAD
Octanoyl-CoA
CoA
CoA
3-Hydroxyoctanoyl-CoA
(2E)-Octenoyl-CoA
H
2
O
H
2
O
NAD
3-Oxooctanoyl-CoA
H
+
NADH
NAD
H
+
NADH
CoA
CoA
Magnesium
FAD
FAD
FAD
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
Mitochondrial
Beta-Oxidation
of Long Chain
Saturated Fatty
Acids
Citric Acid
Cycle
Citric Acid
Cycle
Citric Acid
Cycle
Mitochondrial
Beta-Oxidation
of Short Chain
Saturated Fatty
Acids
Mitochondrial Inner Membrane
Mitochondrial Outer Membrane
Mitochondrial Inner Membrane
Mitochondrial Matrix
Mitochondria
Extracellular Space
Intracellular Space
Mitochondria
SLC25A29
ACSM1
ECHS1
HADHA
HADHB
HADH
HADHA
HADHB
ACAA2
HADHA
HADHB
ECHS1
HADHA
HADHB
HADH
HADHA
HADHB
ACAA2
HADHA
HADHB
ECHS1
HADHA
HADHB
HADH
HADHA
HADHB
ACAA2
HADHA
HADHB
ACADM
ACADM
ACADM
Dodecanoic acid
Lauroyl-CoA
Acetyl-CoA
Acetyl-CoA
Acetyl-CoA
Hexanoyl-CoA
Dodecanoic acid
Adenosine
triphosphate
Coenzyme A
Adenosine
monophosphate
Pyrophosphate
3-
Hydroxydodecanoyl-
CoA
(2E)-
Dodecenoyl- CoA
Water
Water
NAD
3-
Oxododecanoyl-
CoA
NADH
Hydrogen Ion
NAD
NADH
Hydrogen Ion
Decanoyl-CoA
Coenzyme A
Coenzyme A
3-
Hydroxydecanoyl-
CoA
(2E)-
Decenoyl-CoA
Water
Water
NAD
3- Oxodecanoyl-
CoA
Hydrogen Ion
NADH
NAD
Hydrogen Ion
NADH
Octanoyl-CoA
Coenzyme A
Coenzyme A
3-
Hydroxyoctanoyl-
CoA
(2E)-
Octenoyl-CoA
Water
Water
NAD
3- Oxooctanoyl-
CoA
Hydrogen Ion
NADH
NAD
Hydrogen Ion
NADH
Coenzyme A
Coenzyme A
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein