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Glutamine synthetase Glutaminase liver isoform, mitochondrial Glutamine-- fructose-6- phosphate aminotransferase [isomerizing] 1 Glucosamine 6-phosphate N- acetyltransferase N-acetyl-D- glucosamine kinase CAD protein Carbamoyl- phosphate synthase [ammonia], mitochondrial Amidophosphoribosyltransferase GMP synthase [glutamine- hydrolyzing] Glutamine- dependent NAD(+) synthetase Delta-1- pyrroline-5- carboxylate dehydrogenase, mitochondrial Alanine aminotransferase 1 Glutamate dehydrogenase 1, mitochondrial Aspartate aminotransferase, mitochondrial Probable glutamate--tRNA ligase, mitochondrial Glutamate-- cysteine ligase Glutathione synthetase Glutathione reductase, mitochondrial Glutamate decarboxylase 1 4-aminobutyrate aminotransferase, mitochondrial Succinate- semialdehyde dehydrogenase, mitochondrial Glutamine--tRNA ligase L-Glutamic acid Carbamoyl phosphate 5-Phosphoribosylamine Accumulation γ-Aminobutyric acid 1-Pyrroline-5-carboxylic acid ATP NH3 ADP Pi L-Glutamine H2O NH3 Fructose 6-phosphate Glucosamine 6-phosphate L-Glutamic acid Acetyl-CoA N-Acetyl-D-Glucosamine 6-Phosphate CoA ADP Chitin ATP ATP Carbonic acid H2O ADP Pi L-Glutamic acid ATP NH3 CO2 H2O ADP Pi PPi L-Glutamic acid Phosphoribosyl pyrophosphate H2O ATP Xanthylic acid H2O AMP PPi L-Glutamic acid Guanosine monophosphate ATP Nicotinic acid adenine dinucleotide H2O L-Glutamic acid AMP PPi NAD NAD H2O NADH L-Alanine Oxoglutaric acid Pyruvic acid H2O NAD NH3 NADH L-Aspartic acid Oxalacetic acid ATP AMP PPi ATP L-Cysteine γ-Glutamylcysteine ADP Pi ATP Glycine Glutathione ADP Pi Oxidized glutathione NADPH NADP CO2 Oxoglutaric acid Accumulation Succinic acid semialdehyde L-Glutamic acid NAD H2O Succinic acid NADH H+ ATP AMP PPi Biotin Magnesium Zinc (II) ion Magnesium 4Fe-4S tRNA(Glu) L- Glutamyl- tRNA(Glu) tRNA(Gln) L- Glutaminyl- tRNA(Gln) Glutathione Metabolism Pyrimidine Metabolism Purine Metabolism Histidine Metabolism Urea Cycle Arginine and Proline Metabolism Arginine and Proline Metabolism Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Magnesium FAD Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Intracellular Space Extracellular Space Cytosol
PIG59 GLS2 GFPT1 GNPNAT1 NAGK CAD CPS1 PPAT GMPS NADSYN1 ALDH4A1 GPT GLUD1 GOT2 EARS2 GCLC GCLM GSS GSR GAD1 ABAT ALDH5A1 QARS L-Glutamic acid Carbamoyl phosphate 5- Phosphoribosylamine Accumulation γ-Aminobutyric acid 1-Pyrroline- 5-carboxylic acid Adenosine triphosphate Ammonia Adenosine diphosphate Phosphate L-Glutamine Water Ammonia Fructose 6-phosphate Glucosamine 6-phosphate L-Glutamic acid Acetyl-CoA N-Acetyl-D- Glucosamine 6-Phosphate Coenzyme A Adenosine diphosphate ribose Chitin Adenosine triphosphate Adenosine triphosphate Carbonic acid Water Adenosine diphosphate Phosphoric acid L-Glutamic acid Adenosine triphosphate Ammonia Carbon dioxide Water Adenosine diphosphate Phosphoric acid Pyrophosphate L-Glutamic acid Phosphoribosyl pyrophosphate Water Adenosine triphosphate Xanthylic acid Water Adenosine monophosphate Pyrophosphate L-Glutamic acid Guanosine monophosphate Adenosine triphosphate Nicotinic acid adenine dinucleotide Water L-Glutamic acid Adenosine monophosphate Pyrophosphate NAD NAD Water NADH L-Alanine Oxoglutaric acid Pyruvic acid Water NAD Ammonia NADH L-Aspartic acid Oxalacetic acid Adenosine triphosphate Adenosine monophosphate Pyrophosphate Adenosine triphosphate L-Cysteine γ- Glutamylcysteine Adenosine diphosphate ribose Phosphate Adenosine triphosphate Glycine Glutathione Adenosine diphosphate Phosphate Oxidized glutathione NADPH NADP Carbon dioxide Oxoglutaric acid Accumulation Succinic acid semialdehyde L-Glutamic acid NAD Water Succinic acid NADH Hydrogen Ion Adenosine triphosphate Adenosine monophosphate Pyrophosphate tRNA(Glu) L- Glutamyl- tRNA(Glu) tRNA(Gln) L- Glutaminyl- tRNA(Gln) Glutathione Metabolism Pyrimidine Metabolism Purine Metabolism Histidine Metabolism Urea Cycle Arginine and Proline Metabolism Arginine and Proline Metabolism
PIG59 GLS2 GFPT1 GNPNAT1 NAGK CAD CPS1 PPAT GMPS NADSYN1 ALDH4A1 GPT GLUD1 GOT2 EARS2 GCLC GCLM GSS GSR GAD1 ABAT ALDH5A1 QARS Glu CarbmP 5-PPRSA Accumulation GABA 1P5CA ATP NH3 ADP Pi Gln H2O NH3 Fru6P G6P Glu Ac-CoA NAcG6P CoA ADP Chitin ATP ATP CarbncA H2O ADP Pi Glu ATP NH3 CO2 H2O ADP Pi PPi Glu PRPP H2O ATP XntylcA H2O AMP PPi Glu GSMP ATP NctAADn H2O Glu AMP PPi NAD NAD H2O NADH Ala AKG Pyr H2O NAD NH3 NADH Asp Oaa ATP AMP PPi ATP Cys γGluCys ADP Pi ATP Gly GSH ADP Pi GSSG NADPH NADP CO2 AKG Accumulation SuccASA Glu NAD H2O Succini NADH H+ ATP AMP PPi Biotin Mg2+ Zinc Mg2+ 4Fe-4S TRNAGLU LGTG TRNAGLN LGTG PW000004 PW000160 PW000052 PW000043 PW000162 PW000010 PW000010 Pyr-5'P Pyr-5'P Mg2+ FAD Pyr-5'P Pyr-5'P Intracellular Space Extracellular Space Cytosol
PIG59 GLS2 GFPT1 GNPNAT1 NAGK CAD CPS1 PPAT GMPS NADSYN1 ALDH4A1 GPT GLUD1 GOT2 EARS2 GCLC GCLM GSS GSR GAD1 ABAT ALDH5A1 QARS Glu CarbmP 5-PPRSA Accumulation GABA 1P5CA ATP NH3 ADP Pi Gln H2O NH3 Fru6P G6P Glu Ac-CoA NAcG6P CoA ADP-Rib Chitin ATP ATP CarbncA H2O ADP PhosfrA Glu ATP NH3 CO2 H2O ADP PhosfrA Ppi Glu PRPP H2O ATP XntylcA H2O AMP Ppi Glu GSMP ATP NctAADn H2O Glu AMP Ppi NAD NAD H2O NADH Ala AKG Pyr H2O NAD NH3 NADH Asp Oaa ATP AMP Ppi ATP Cys γGluCys ADP-Rib Pi ATP Gly GSH ADP Pi GSSG NADPH NADP CO2 AKG Accumulation SuccASA Glu NAD H2O Succini NADH H+ ATP AMP Ppi TRNAGLU LGTG TRNAGLN LGTG PW000004 PW000160 PW000052 PW000043 PW000162 PW000010 PW000010