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Neutral amino
acid
transporter
B(0)
Neutral amino
acid
transporter A
Sodium-coupled
neutral amino
acid
transporter 1
Alanine
aminotransferase
1
Glutaminase
liver isoform,
mitochondrial
Glutamate
dehydrogenase
1,
mitochondrial
Carbamoyl-
phosphate
synthase
[ammonia],
mitochondrial
Aspartate
aminotransferase,
mitochondrial
Ornithine
carbamoyltransferase,
mitochondrial
Arginase-1
Argininosuccinate
lyase
Argininosuccinate
synthase
L-Glutamine
L-Alanine
Oxoglutaric acid
L-Glutamic acid
Oxalacetic acid
NH
3
L-Aspartic acid
Fumaric acid
Ornithine
L-Glutamine
L-Alanine
Ornithine
Citrulline
L-Aspartic acid
L-Glutamic acid
L-Glutamic acid
Argininosuccinic acid
Citrulline
Pyruvic acid
H
2
O
H
2
O
NAD
Oxoglutaric acid
NADH
ATP
CO
2
H
2
O
ADP
P
i
Carbamoyl phosphate
P
i
H
2
O
Urea
ATP
AMP
PP
i
L-Arginine
Citrulline
Y+L amino acid
transporter 1
Argininosuccinic acid
4F2
cell-surface
antigen heavy
chain
Ammonia
Recycling
Pyrimidine
Metabolism
Glutamate
Metabolism
Alanine
Metabolism
Glucose-Alanine
Cycle
Selenoamino
Acid Metabolism
Malate-
Aspartate
Shuttle
Glutamate
Metabolism
Alanine
Metabolism
Glucose-Alanine
Cycle
Gluconeogenesis
Histidine
Metabolism
Porphyrin
Metabolism
Glucose-Alanine
Cycle
Porphyrin
Metabolism
Alanine
Metabolism
Ammonia
Recycling
Glutamate
Metabolism
Glucose-Alanine
Cycle
Ammonia
Recycling
Purine
Metabolism
Glucose-Alanine
Cycle
Aspartate
Metabolism
Citric Acid
Cycle
Arginine and
Proline
Metabolism
D-Arginine and
D-Ornithine
Metabolism
D-Arginine and
D-Ornithine
Metabolism
Mitochondrial
ornithine
transporter 1
Mitochondrial
ornithine
transporter 1
Calcium-binding
mitochondrial
carrier protein
Aralar1
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
Manganese
Calcium
Cytosol
Mitochondria
Mitochondrial Matrix
Urea Cycle
Extracellular Space
Intracellular Space
Infants are lethargic and
unwilling to eat. They may
develop seizures, coma, and
failure to thrive as toxic
ammonia accumulates.
Mitochondria
SLC1A5
SLC1A4
SLC38A1
GPT
GLS2
GLUD1
CPS1
GOT2
OTC
ARG1
ASL
ASS1
L-Glutamine
L-Alanine
Oxoglutaric
acid
L-Glutamic acid
Oxalacetic acid
Ammonia
L-Aspartic acid
Fumaric acid
Ornithine
L-Glutamine
L-Alanine
Ornithine
Citrulline
L-Aspartic acid
L-Glutamic acid
L-Glutamic acid
Argininosuccinic
acid
Citrulline
Pyruvic acid
Water
Water
NAD
Oxoglutaric
acid
NADH
Adenosine
triphosphate
Carbon dioxide
Water
Adenosine
diphosphate
Phosphoric acid
Carbamoyl
phosphate
Phosphate
Water
Urea
Adenosine
triphosphate
Adenosine
monophosphate
Pyrophosphate
L-Arginine
Citrulline
SLC7A7
Argininosuccinic
acid
SLC3A2
Ammonia
Recycling
Pyrimidine
Metabolism
Glutamate
Metabolism
Alanine
Metabolism
Glucose-Alanine
Cycle
Selenoamino
Acid Metabolism
Malate-
Aspartate
Shuttle
Glutamate
Metabolism
Alanine
Metabolism
Glucose-Alanine
Cycle
Gluconeogenesis
Histidine
Metabolism
Porphyrin
Metabolism
Glucose-Alanine
Cycle
Porphyrin
Metabolism
Alanine
Metabolism
Ammonia
Recycling
Glutamate
Metabolism
Glucose-Alanine
Cycle
Ammonia
Recycling
Purine
Metabolism
Glucose-Alanine
Cycle
Aspartate
Metabolism
Citric Acid
Cycle
Arginine and
Proline
Metabolism
D-Arginine and
D-Ornithine
Metabolism
D-Arginine and
D-Ornithine
Metabolism
SLC25A15
SLC25A15
SLC25A12