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Neutral amino acid transporter B(0) Neutral amino acid transporter A Ammonium transporter Rh type A 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 L-Arginine Ornithine L-Glutamine L-Alanine Ornithine Citrulline Citrulline L-Aspartic acid L-Glutamic acid L-Glutamic acid NH3 Pyruvic acid H2O H2O NAD Oxoglutaric acid NADH ATP CO2 H2O ADP Pi Carbamoyl phosphate Pi H2O Urea Argininosuccinic acid ATP AMP PPi 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 Symptoms of the disorder include lethargy, seizures, or coma.
Mitochondria SLC1A5 SLC1A4 RHAG 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 L-Arginine Ornithine L-Glutamine L-Alanine Ornithine Citrulline Citrulline L-Aspartic acid L-Glutamic acid L-Glutamic acid Ammonia Pyruvic acid Water Water NAD Oxoglutaric acid NADH Adenosine triphosphate Carbon dioxide Water Adenosine diphosphate Phosphoric acid Carbamoyl phosphate Phosphate Water Urea Argininosuccinic acid Adenosine triphosphate Adenosine monophosphate Pyrophosphate 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