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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 L-Arginine Ornithine L-Glutamine L-Alanine Ornithine Citrulline Citrulline L-Aspartic acid L-Glutamic acid L-Glutamic acid Citrulline 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 vomiting, lethargy and intracranial pressure.
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 L-Arginine Ornithine L-Glutamine L-Alanine Ornithine Citrulline Citrulline L-Aspartic acid L-Glutamic acid L-Glutamic acid Citrulline 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
SLC1A5 SLC1A4 SLC38A1 GPT GLS2 GLUD1 CPS1 GOT2 OTC ARG1 ASL ASS1 Gln Ala AKG Glu Oaa NH3 Asp Fumarat Arg Ornithi Gln Ala Ornithi Citruln Citruln Asp Glu Glu Citruln Pyr H2O H2O NAD AKG NADH ATP CO2 H2O ADP Pi CarbmP Pi H2O Urea 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 Symptoms of the disorder include vomiting, lethargy and intracranial pressure.
Mitochondria SLC1A5 SLC1A4 SLC38A1 GPT GLS2 GLUD1 CPS1 GOT2 OTC ARG1 ASL ASS1 Gln Ala AKG Glu Oaa NH3 Asp Fumarat Arg Ornithi Gln Ala Ornithi Citruln Citruln Asp Glu Glu Citruln Pyr H2O H2O NAD AKG NADH ATP CO2 H2O ADP PhosfrA CarbmP Pi H2O Urea 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