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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 NH3 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 H2O H2O NAD Oxoglutaric acid NADH ATP CO2 H2O ADP Pi Carbamoyl phosphate Pi H2O Urea ATP AMP PPi 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