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Neutral amino acid transporter B(0) Neutral amino acid transporter 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 Accumulation L-Glutamine Accumulation L-Alanine Oxoglutaric acid L-Glutamic acid Oxalacetic acid Accumulation NH3 L-Aspartic acid Fumaric acid L-Arginine Ornithine Accumulation L-Glutamine Accumulation L-Alanine Ornithine Accumulation Citrulline Accumulation Citrulline L-Aspartic acid L-Glutamic acid L-Glutamic acid Pyruvic acid H2O H2O NAD Oxoglutaric acid NADH ATP CO2 H2O ADP Pi Carbamoyl phosphate Pi H2O Urea Dissipation 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
Mitochondria SLC1A5 SLC1A4 GPT GLS2 GLUD1 CPS1 GOT2 OTC ARG1 ASL ASS1 Accumulation L-Glutamine Accumulation L-Alanine Oxoglutaric acid L-Glutamic acid Oxalacetic acid Accumulation Ammonia L-Aspartic acid Fumaric acid L-Arginine Ornithine Accumulation L-Glutamine Accumulation L-Alanine Ornithine Accumulation Citrulline Accumulation Citrulline L-Aspartic acid L-Glutamic acid L-Glutamic acid Pyruvic acid Water Water NAD Oxoglutaric acid NADH Adenosine triphosphate Carbon dioxide Water Adenosine diphosphate Phosphoric acid Carbamoyl phosphate Phosphate Water Urea Dissipation 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
SLC1A5 SLC1A4 GPT GLS2 GLUD1 CPS1 GOT2 OTC ARG1 ASL ASS1 Accumulation Gln Accumulation Ala AKG Glu Oaa Accumulation NH3 Asp Fumarat Arg Ornithi Accumulation Gln Accumulation Ala Ornithi Accumulation Citruln Accumulation Citruln Asp Glu Glu Pyr H2O H2O NAD AKG NADH ATP CO2 H2O ADP Pi CarbmP Pi H2O Urea Dissipation ASA ATP AMP PPi PW000009 PW000160 PW000003 PW000001 PW000046 PW000007 PW000030 PW000003 PW000001 PW000046 PW000152 PW000043 PW000158 PW000046 PW000158 PW000001 PW000009 PW000003 PW000046 PW000009 PW000052 PW000046 PW000002 PW000005 PW000010 PW000019 PW000019 SLC25A15 SLC25A15 SLC25A12 Pyr-5'P Pyr-5'P Mn2+ Ca2+ Cytosol Mitochondria Mitochondrial Matrix Urea Cycle Extracellular Space Intracellular Space
Mitochondria SLC1A5 SLC1A4 GPT GLS2 GLUD1 CPS1 GOT2 OTC ARG1 ASL ASS1 Accumulation Gln Accumulation Ala AKG Glu Oaa Accumulation NH3 Asp Fumarat Arg Ornithi Accumulation Gln Accumulation Ala Ornithi Accumulation Citruln Accumulation Citruln Asp Glu Glu Pyr H2O H2O NAD AKG NADH ATP CO2 H2O ADP PhosfrA CarbmP Pi H2O Urea Dissipation ASA ATP AMP Ppi PW000009 PW000160 PW000003 PW000001 PW000046 PW000007 PW000030 PW000003 PW000001 PW000046 PW000152 PW000043 PW000158 PW000046 PW000158 PW000001 PW000009 PW000003 PW000046 PW000009 PW000052 PW000046 PW000002 PW000005 PW000010 PW000019 PW000019 SLC25A15 SLC25A15 SLC25A12