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Mitochondrial
ornithine
transporter 1
Argininosuccinate
synthase
Argininosuccinate
lyase
Carbamoyl-
phosphate
synthase
[ammonia],
mitochondrial
Glutamate
dehydrogenase
1,
mitochondrial
Ornithine
aminotransferase,
mitochondrial
Delta-1-
pyrroline-5-
carboxylate
dehydrogenase,
mitochondrial
Delta-1-
pyrroline-5-
carboxylate
dehydrogenase,
mitochondrial
Hydroxyproline
dehydrogenase
Pyrroline-5-
carboxylate
reductase 2
Prolyl
4-hydroxylase
subunit alpha-3
Hydroxyproline
dehydrogenase
Pyrroline-5-
carboxylate
reductase 2
Arginyl-tRNA
synthetase 2,
mitochondrial
Glycine
amidinotransferase,
mitochondrial
Guanidinoacetate
N-
methyltransferase
Creatine kinase
B-type
Arginase-1
Ornithine
carbamoyltransferase,
mitochondrial
D-amino-acid
oxidase
D-amino-acid
oxidase
Delta-1-
pyrroline-5-
carboxylate
dehydrogenase,
mitochondrial
Hydroxyproline
dehydrogenase
Delta-1-
pyrroline-5-
carboxylate
dehydrogenase,
mitochondrial
Aspartate
aminotransferase,
cytoplasmic
Nitric oxide
synthase, brain
Glutamyl-
prolyl-tRNA
synthetase
Fumaric acid
L-Aspartic acid
Ornithine
L-Arginine
Ornithine
Citrulline
Citrulline
ATP
AMP
PPi
Argininosuccinic acid
ATP
NH3
CO2
H2 O
ADP
Phosphoric acid
Carbamoyl
phosphate
L-Glutamic acid
H2 O
NAD
Oxoglutaric
acid
NADH
Oxoglutaric
acid
L-Glutamic γ-semialdehyde
NAD
H2 O
NADH
H+
1-Pyrroline-5-carboxylic acid
NAD
H2 O
NADH
L-Proline
NAD
NADH
Oxoglutaric
acid
O2
4-
Hydroxyproline
Succinic acid
CO2
Pyrroline
hydroxycarboxylic
acid
NAD
NADH
ATP
AMP
PPi
Glycine
Ornithine
Dissipation
Guanidoacetic
acid
S-Adenosylmethionine
S-Adenosylhomocysteine
Dissipation
Creatine
ATP
ADP
Phosphocreatine
H2 O
Urea
Pi
D-Proline
H2 O
O2
1-Pyrroline-
2-carboxylic
acid
NH3
H2 O2
H2 O
O2
1-Pyrroline-
4-hydroxy-2-
carboxylate
NH3
H2 O2
NAD
H2 O
4-Hydroxy-L-
glutamic acid
NADPH
L-4-
Hydroxyglutamate
semialdehyde
NAD
H2 O
NADPH
Oxalacetic acid
4-Hydroxy-2-
oxoglutaric
acid
L-Aspartic acid
NADPH
O2
N(omega)-Hydroxyarginine
Nitric oxide
NADP
H2 O
ATP
AMP
PPi
Pyridoxal
5'-phosphate
FAD
FAD
Manganese
FAD
FAD
FAD
Pyridoxal
5'-phosphate
Heme
FAD
Flavin
Mononucleotide
Tetrahydrobiopterin
tRNA(Arg)
L-Arginyl-
tRNA(Arg)
tRNA(Pro)
L-Prolyl-
tRNA(Pro)
Citric Acid
Cycle
Alanine
Metabolism
D-Arginine and
D-Ornithine
Metabolism
D-Arginine and
D-Ornithine
Metabolism
Urea Cycle
Intracellular Space
Extracellular Space
Mitochondria
Lysosome
Mitochondria
Mitochondria
Slc25a15
Ass1
Asl
Cps1
Glud1
Oat
Aldh4a1
Aldh4a1
Prodh2
Pycr2
P4ha3
Prodh2
Pycr2
Rars2
Gatm
Gamt
Ckb
Arg1
Otc
Dao
Dao
Aldh4a1
Prodh2
Aldh4a1
Got1
Nos1
Eprs
Fumaric acid
L-Aspartic acid
Ornithine
L-Arginine
Ornithine
Citrulline
Citrulline
Adenosine
triphosphate
Adenosine
monophosphate
Pyrophosphate
Argininosuccinic
acid
Adenosine
triphosphate
Ammonia
Carbon dioxide
Water
Adenosine
diphosphate
Phosphoric acid
Carbamoyl
phosphate
L-Glutamic acid
Water
NAD
Oxoglutaric
acid
NADH
Oxoglutaric
acid
L-Glutamic
γ-semialdehyde
NAD
Water
NADH
Hydrogen Ion
1-Pyrroline-
5-carboxylic
acid
NAD
Water
NADH
L-Proline
NAD
NADH
Oxoglutaric
acid
Oxygen
4-
Hydroxyproline
Succinic acid
Carbon dioxide
Pyrroline
hydroxycarboxylic
acid
NAD
NADH
Adenosine
triphosphate
Adenosine
monophosphate
Pyrophosphate
Glycine
Ornithine
Dissipation
Guanidoacetic
acid
S-
Adenosylmethionine
S-
Adenosylhomocysteine
Dissipation
Creatine
Adenosine
triphosphate
Adenosine
diphosphate
Phosphocreatine
Water
Urea
Phosphate
D-Proline
Water
Oxygen
1-Pyrroline-
2-carboxylic
acid
Ammonia
Hydrogen
peroxide
Water
Oxygen
1-Pyrroline-
4-hydroxy-2-
carboxylate
Ammonia
Hydrogen
peroxide
NAD
Water
4-Hydroxy-L-
glutamic acid
NADPH
L-4-
Hydroxyglutamate
semialdehyde
NAD
Water
NADPH
Oxalacetic acid
4-Hydroxy-2-
oxoglutaric
acid
L-Aspartic acid
NADPH
Oxygen
N(omega)-
Hydroxyarginine
Nitric oxide
NADP
Water
Adenosine
triphosphate
Adenosine
monophosphate
Pyrophosphate
tRNA(Arg)
L-Arginyl-
tRNA(Arg)
tRNA(Pro)
L-Prolyl-
tRNA(Pro)
Citric Acid
Cycle
Alanine
Metabolism
D-Arginine and
D-Ornithine
Metabolism
D-Arginine and
D-Ornithine
Metabolism
Slc25a15
Ass1
Asl
Cps1
Glud1
Oat
Aldh4a1
Aldh4a1
Prodh2
Pycr2
P4ha3
Prodh2
Pycr2
Rars2
Gatm
Gamt
Ckb
Arg1
Otc
Dao
Dao
Aldh4a1
Prodh2
Aldh4a1
Got1
Nos1
Eprs
Fumarat
Asp
Ornithi
Arg
Ornithi
Citruln
Citruln
ATP
AMP
PPi
ASA
ATP
NH3
CO2
H2 O
ADP
PhosfrA
CarbmP
Glu
H2 O
NAD
AKG
NADH
AKG
L-Gl-GA
NAD
H2 O
NADH
H+
1P5CA
NAD
H2 O
NADH
Pro
NAD
NADH
AKG
O2
4-HPro
Succini
CO2
PyroHcA
NAD
NADH
ATP
AMP
PPi
Gly
Ornithi
Dissipation
GIAA
SAMe
SAH
Dissipation
Creatin
ATP
ADP
Phosfcr
H2 O
Urea
Pi
D-Pro
H2 O
O2
1Pyr2C
NH3
H2 O2
H2 O
O2
1P4H2C
NH3
H2 O2
NAD
H2 O
4HLG
NADPH
44H5OLN
NAD
H2 O
NADPH
Oaa
2O4H
Asp
NADPH
O2
NωH
NO
NADP
H2 O
ATP
AMP
PPi
Pyr-5'P
FAD
FAD
Mn2+
FAD
FAD
FAD
Pyr-5'P
Heme
FAD
FlvnMnt
BH4
TRNAARG
LATA
TRNAPRO
LPTP
PW000005
PW000001
PW000019
PW000019
Urea Cycle
Intracellular Space
Extracellular Space
Mitochondria
Lysosome
Mitochondria
Mitochondria
Slc25a15
Ass1
Asl
Cps1
Glud1
Oat
Aldh4a1
Aldh4a1
Prodh2
Pycr2
P4ha3
Prodh2
Pycr2
Rars2
Gatm
Gamt
Ckb
Arg1
Otc
Dao
Dao
Aldh4a1
Prodh2
Aldh4a1
Got1
Nos1
Eprs
Fumarat
Asp
Ornithi
Arg
Ornithi
Citruln
Citruln
ATP
AMP
Ppi
ASA
ATP
NH3
CO2
H2O
ADP
PhosfrA
CarbmP
Glu
H2O
NAD
AKG
NADH
AKG
L-Gl-GA
NAD
H2O
NADH
H+
1P5CA
NAD
H2O
NADH
Pro
NAD
NADH
AKG
O2
4-HPro
Succini
CO2
PyroHcA
NAD
NADH
ATP
AMP
Ppi
Gly
Ornithi
Dissipation
GIAA
SAMe
SAH
Dissipation
Creatin
ATP
ADP
Phosfcr
H2O
Urea
Pi
D-Pro
H2O
O2
1Pyr2C
NH3
H2O2
H2O
O2
1P4H2C
NH3
H2O2
NAD
H2O
4HLG
NADPH
44H5OLN
NAD
H2O
NADPH
Oaa
2O4H
Asp
NADPH
O2
NωH
NO
NADP
H2O
ATP
AMP
Ppi
TRNAARG
LATA
TRNAPRO
LPTP
PW000005
PW000001
PW000019
PW000019