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Low affinity
cationic amino
acid
transporter 2
Mitochondrial
ornithine
transporter 2
Unknown
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
Peroxisomal
sarcosine
oxidase
Glutaryl-CoA
dehydrogenase,
mitochondrial
Oxoglutarate
dehydrogenase
complex
Acetyl-CoA
L-Lysine
L-Lysine
L-Lysine
Allysine
Allysine
NADPH
Oxoglutaric acid
Saccharopine
NADP
H
2
O
H
2
O
NAD
L-Glutamic acid
NADH
NAD
H
2
O
Accumulation
Aminoadipic acid
NADH
Oxoglutaric acid
Accumulation
Oxoadipic acid
L-Glutamic acid
3-Hydroxybutyryl-CoA
Crotonoyl-CoA
H
2
O
NAD
Acetoacetyl-CoA
NADH
CoA
L-Pipecolic acid
O
2
(S)-2,3,4,5-
Tetrahydropiperidine-2-
carboxylate
H
2
O
2
Glutaryl-CoA
CO
2
CoA
H
2
O
Pyridoxal
5'-phosphate
Thiamine
pyrophosphate
Lipoamide
FAD
Citric Acid
Cycle
Biotin
Metabolism
FAD
FAD
Inner Mitochondria Membrane
Outer Mitochondria Membrane
Mitochondrial Intermembrane
Space
Peroxisome
Mitochondria
Extracellular Space
Intracellular Space
Liver
Extracellular Space
Mitochondrial matrix
Liver Hepatic Cell
Mitochondria
SLC7A2
SLC25A2
Unknown
AASS
AASS
ALDH7A1
AADAT
ECHS1
HADH
ACAT1
PIPOX
GCDH
DHTKD1
Acetyl-CoA
L-Lysine
L-Lysine
L-Lysine
Allysine
Allysine
NADPH
Oxoglutaric
acid
Saccharopine
NADP
Water
Water
NAD
L-Glutamic acid
NADH
NAD
Water
Accumulation
Aminoadipic
acid
NADH
Oxoglutaric
acid
Accumulation
Oxoadipic acid
L-Glutamic acid
3-
Hydroxybutyryl-
CoA
Crotonoyl-CoA
Water
NAD
Acetoacetyl-CoA
NADH
Coenzyme A
L-Pipecolic
acid
Oxygen
(S)-2,3,4,5-
Tetrahydropiperidine-
2-carboxylate
Hydrogen
peroxide
Glutaryl-CoA
Carbon dioxide
Coenzyme A
Water
Citric Acid
Cycle
Biotin
Metabolism