Cationic amino
acid
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
NADPH
Oxoglutaric
acid
Saccharopine
NADP
H2O
H2O
NAD
Allysine
L-Glutamic acid
NADH
NAD
H2O
Accumulation
Aminoadipic acid
NADH
Oxoglutaric acid
Accumulation
Oxoadipic acid
L-Glutamic acid
3-Hydroxybutyryl-CoA
Crotonoyl-CoA
H2O
NAD
Acetoacetyl-CoA
NADH
CoA
(S)-2,3,4,5-
Tetrahydropiperidine-2-
carboxylate
H2O
L-Pipecolic acid
O2
H2O2
Glutaryl-CoA
CO2
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