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Cationic amino
acid
transporter 2
Mitochondrial
ornithine
transporter 1
Alpha-
aminoadipic
semialdehyde
synthase,
mitochondrial
Alpha-
aminoadipic
semialdehyde
synthase,
mitochondrial
Alpha-
aminoadipic
semialdehyde
dehydrogenase
Kynurenine/alpha-
aminoadipate
aminotransferase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Hydroxyacyl-
coenzyme A
dehydrogenase,
mitochondrial
Acetyl-CoA
acetyltransferase,
mitochondrial
Pipecolic acid
and sarcosine
oxidase
Short-chain
specific
acyl-CoA
dehydrogenase,
mitochondrial
Oxoglutarate
dehydrogenase
complex
Acetyl-CoA
L-Lysine
L-Lysine
L-Lysine
NADPH
Oxoglutaric
acid
Saccharopine
NADP
H
2
O
H
2
O
NAD
Allysine
L-Glutamic acid
NADH
NAD
H
2
O
Aminoadipic acid
NADH
Oxoglutaric acid
Oxoadipic acid
L-Glutamic acid
3-Hydroxybutyryl-CoA
Crotonoyl-CoA
H
2
O
NAD
Acetoacetyl-CoA
NADH
CoA
(S)-2,3,4,5-
Tetrahydropiperidine-2-
carboxylate
H
2
O
L-Pipecolic acid
O
2
H
2
O
2
Glutaryl-CoA
CO
2
CoA
Pyridoxal
5'-phosphate
FAD
FAD
Thiamine
pyrophosphate
Lipoamide
FAD
Citric Acid
Cycle
Biotin
Metabolism
Inner Mitochondria Membrane
Outer Mitochondria Membrane
Mitochondrial Intermembrane
Space
Peroxisome
Mitochondria
Extracellular Space
Intracellular Space
Liver
Extracellular Space
Mitochondrial matrix
Mitochondria
Slc7a2
Slc25a15
Aass
Aass
Aldh7a1
Aadat
Echs1
Hadh
Acat1
Pipox
Acads
Dhtkd1
Acetyl-CoA
L-Lysine
L-Lysine
L-Lysine
NADPH
Oxoglutaric
acid
Saccharopine
NADP
Water
Water
NAD
Allysine
L-Glutamic acid
NADH
NAD
Water
Aminoadipic
acid
NADH
Oxoglutaric
acid
Oxoadipic acid
L-Glutamic acid
3-
Hydroxybutyryl-
CoA
Crotonoyl-CoA
Water
NAD
Acetoacetyl-CoA
NADH
Coenzyme A
(S)-2,3,4,5-
Tetrahydropiperidine-
2-carboxylate
Water
L-Pipecolic
acid
Oxygen
Hydrogen
peroxide
Glutaryl-CoA
Carbon dioxide
Coenzyme A
Citric Acid
Cycle
Biotin
Metabolism