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Carnitine O-
palmitoyltransferase
2,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
3-ketoacyl-CoA
thiolase,
mitochondrial
Carnitine O-
palmitoyltransferase
1, liver
isoform
Long-chain-
fatty-acid--
CoA ligase 1
Enoyl-CoA
hydratase,
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
3-ketoacyl-CoA
thiolase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
3-ketoacyl-CoA
thiolase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
3-ketoacyl-CoA
thiolase,
mitochondrial
Long-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Short/branched
chain specific
acyl-CoA
dehydrogenase,
mitochondrial
Short-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
3-ketoacyl-CoA
thiolase,
mitochondrial
Long-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Short/branched
chain specific
acyl-CoA
dehydrogenase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Acetyl-CoA
acetyltransferase,
mitochondrial
Glutaryl-CoA
dehydrogenase,
mitochondrial
Very long-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Long-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Long-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Long-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Long-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Long-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Trifunctional
enzyme subunit
alpha,
mitochondrial
Trifunctional
enzyme subunit
beta,
mitochondrial
Palmitic acid
Accumulation
L-Palmitoylcarnitine
Accumulation
L-Palmitoylcarnitine
Palmitoyl-CoA
L-Carnitine
CoA
3-Hydroxyhexadecanoyl-CoA
(2E)-Hexadecenoyl-CoA
H2 O
NAD
3-oxohexadecanoyl-CoA
NADH
H+
Tetradecanoyl-CoA
Acetyl-CoA
CoA
Palmitoyl-CoA
L-Carnitine
CoA
ATP
CoA
AMP
PPi
3-Hydroxytetradecanoyl-CoA
(2E)-Tetradecenoyl-CoA
H2 O
NAD
3-oxotetradecanoyl-CoA
NADH
H+
Lauroyl-CoA
Acetyl-CoA
CoA
3-Hydroxydodecanoyl-CoA
NAD
3-oxododecanoyl-CoA
NADH
H+
Decanoyl-CoA
Acetyl-CoA
CoA
3-Hydroxydecanoyl-CoA
(2E)-Decenoyl-CoA
H2 O
NAD
3-oxodecanoyl-CoA
H+
NADH
Octanoyl-CoA
Acetyl-CoA
CoA
3-hydroxyoctanoyl-CoA
(2E)-Octenoyl-CoA
H2 O
NAD
3-oxooctanoyl-CoA
H+
NADH
Acetyl-CoA
Hexanoyl-CoA
CoA
(2E)-Hexenoyl-CoA
3-Hydroxyhexanoyl-CoA
H2 O
NAD
3-oxohexanoyl-CoA
NADH
H+
Acetyl-CoA
Butyryl-CoA
CoA
Crotonoyl-CoA
3-Hydroxybutyryl-CoA
H2 O
Acetyl-CoA
Acetoacetyl-CoA
CoA
Glutaryl-CoA
CO2
NAD
NADH
H+
(2E)-Tetradecenoyl-CoA
H2 O
Magnesium
FAD
FAD
FAD
FAD
FAD
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
Fatty Acid
Elongation in
Mitochondria
Glycerolipid
Metabolism
Phospholipid
Biosynthesis
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
Mitochondrial Matrix
Inner Mitochondria Membrane
Mitochondrial Intermembrane
Space
Outer Mitochondria Membrane
Cytosol
Extracellular Space
Mitochondria
CPT2
ECHS1
HADHA
HADHB
ACAA2
CPT1A
ACSL1
ECHS1
HADHA
HADHB
ACAA2
HADHA
HADHB
ACAA2
ECHS1
HADHA
HADHB
ACAA2
ECHS1
HADHA
HADHB
ACAA2
ACADL
ACADM
ACADSB
ACADS
ECHS1
HADHA
HADHB
ACAA2
ACADL
ACADM
ACADSB
ECHS1
ACAT1
GCDH
ACADVL
ACADL
ACADM
ACADL
ACADM
ACADL
ACADM
ECHS1
ACADL
ACADM
ACADL
ACADM
HADHA
HADHB
Palmitic acid
Accumulation
L-
Palmitoylcarnitine
Accumulation
L-
Palmitoylcarnitine
Palmitoyl-CoA
L-Carnitine
Coenzyme A
3-
Hydroxyhexadecanoyl-
CoA
(2E)-
Hexadecenoyl-
CoA
Water
NAD
3-
oxohexadecanoyl-
CoA
NADH
Hydrogen Ion
Tetradecanoyl-
CoA
Acetyl-CoA
Coenzyme A
Palmitoyl-CoA
L-Carnitine
Coenzyme A
Adenosine
triphosphate
Coenzyme A
Adenosine
monophosphate
Pyrophosphate
3-
Hydroxytetradecanoyl-
CoA
(2E)-
Tetradecenoyl-
CoA
Water
NAD
3-
oxotetradecanoyl-
CoA
NADH
Hydrogen Ion
Lauroyl-CoA
Acetyl-CoA
Coenzyme A
3-
Hydroxydodecanoyl-
CoA
NAD
3-
oxododecanoyl-
CoA
NADH
Hydrogen Ion
Decanoyl-CoA
Acetyl-CoA
Coenzyme A
3-
Hydroxydecanoyl-
CoA
(2E)-
Decenoyl-CoA
Water
NAD
3- oxodecanoyl-
CoA
Hydrogen Ion
NADH
Octanoyl-CoA
Acetyl-CoA
Coenzyme A
3-
hydroxyoctanoyl-
CoA
(2E)-
Octenoyl-CoA
Water
NAD
3- oxooctanoyl-
CoA
Hydrogen Ion
NADH
Acetyl-CoA
Hexanoyl-CoA
Coenzyme A
(2E)-
Hexenoyl-CoA
3-
Hydroxyhexanoyl-
CoA
Water
NAD
3- oxohexanoyl-
CoA
NADH
Hydrogen Ion
Acetyl-CoA
Butyryl-CoA
Coenzyme A
Crotonoyl-CoA
3-
Hydroxybutyryl-
CoA
Water
Acetyl-CoA
Acetoacetyl-CoA
Coenzyme A
Glutaryl-CoA
Carbon dioxide
NAD
NADH
Hydrogen Ion
(2E)-
Tetradecenoyl-
CoA
Water
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
electron-
transfer
flavoprotein
Reduced
electron-
transfer
flavoprotein
Fatty Acid
Elongation in
Mitochondria
Glycerolipid
Metabolism
Phospholipid
Biosynthesis
CPT2
ECHS1
HADHA
HADHB
ACAA2
CPT1A
ACSL1
ECHS1
HADHA
HADHB
ACAA2
HADHA
HADHB
ACAA2
ECHS1
HADHA
HADHB
ACAA2
ECHS1
HADHA
HADHB
ACAA2
ACADL
ACADM
ACADSB
ACADS
ECHS1
HADHA
HADHB
ACAA2
ACADL
ACADM
ACADSB
ECHS1
ACAT1
GCDH
ACADVL
ACADL
ACADM
ACADL
ACADM
ACADL
ACADM
ECHS1
ACADL
ACADM
ACADL
ACADM
HADHA
HADHB
16:0
Accumulation
L-Plmtc
Accumulation
L-Plmtc
COA16:0
L-Carnt
CoA
BHC
(E)-C16
H2 O
NAD
3OHDCoA
NADH
H+
COA14:0
Ac-CoA
CoA
COA16:0
L-Carnt
CoA
ATP
CoA
AMP
PPi
S3HTCoA
2TCoA
H2 O
NAD
3OTDCoA
NADH
H+
COA12:0
Ac-CoA
CoA
S3HDCoA
NAD
3ODDCoA
NADH
H+
Decanoy
Ac-CoA
CoA
SHD-CoA
2DCoA
H2 O
NAD
3ODCCoA
H+
NADH
COA8:0
Ac-CoA
CoA
S-HOCoA
2OCoA
H2 O
NAD
3OOACoA
H+
NADH
Ac-CoA
COA6:0
CoA
T2HCoA
S-HHxCA
H2 O
NAD
3OHOCoA
NADH
H+
Ac-CoA
Btyl-CA
CoA
CrtylCA
3HB-CoA
H2 O
Ac-CoA
ActaCoA
CoA
GlutCoA
CO2
NAD
NADH
H+
2TCoA
H2 O
Mg2+
FAD
FAD
FAD
FAD
FAD
ETF
RETF
ETF
RETF
ETF
RETF
ETF
RETF
ETF
RETF
ETF
RETF
PW000022
PW000144
PW000048
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
FAD
Mitochondrial Matrix
Inner Mitochondria Membrane
Mitochondrial Intermembrane
Space
Outer Mitochondria Membrane
Cytosol
Extracellular Space
Mitochondria
CPT2
ECHS1
HADHA
HADHB
ACAA2
CPT1A
ACSL1
ECHS1
HADHA
HADHB
ACAA2
HADHA
HADHB
ACAA2
ECHS1
HADHA
HADHB
ACAA2
ECHS1
HADHA
HADHB
ACAA2
ACADL
ACADM
ACADSB
ACADS
ECHS1
HADHA
HADHB
ACAA2
ACADL
ACADM
ACADSB
ECHS1
ACAT1
GCDH
ACADVL
ACADL
ACADM
ACADL
ACADM
ACADL
ACADM
ECHS1
ACADL
ACADM
ACADL
ACADM
HADHA
HADHB
16:0
Accumulation
L-Plmtc
Accumulation
L-Plmtc
COA16:0
L-Carnt
CoA
BHC
(E)-C16
H2O
NAD
3OHDCoA
NADH
H+
COA14:0
Ac-CoA
CoA
COA16:0
L-Carnt
CoA
ATP
CoA
AMP
Ppi
S3HTCoA
2TCoA
H2O
NAD
3OTDCoA
NADH
H+
COA12:0
Ac-CoA
CoA
S3HDCoA
NAD
3ODDCoA
NADH
H+
Decanoy
Ac-CoA
CoA
SHD-CoA
2DCoA
H2O
NAD
3ODCCoA
H+
NADH
COA8:0
Ac-CoA
CoA
S-HOCoA
2OCoA
H2O
NAD
3OOACoA
H+
NADH
Ac-CoA
COA6:0
CoA
T2HCoA
S-HHxCA
H2O
NAD
3OHOCoA
NADH
H+
Ac-CoA
Btyl-CA
CoA
CrtylCA
3HB-CoA
H2O
Ac-CoA
ActaCoA
CoA
GlutCoA
CO2
NAD
NADH
H+
2TCoA
H2O
ETF
RETF
ETF
RETF
ETF
RETF
ETF
RETF
ETF
RETF
ETF
RETF
PW000022
PW000144
PW000048