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Glycine cleavage system H protein, mitochondrial L-serine dehydratase/L- threonine deaminase Isoaspartyl peptidase/L- asparaginase Asparagine synthetase [glutamine- hydrolyzing] Histidine ammonia-lyase Carbamoyl- phosphate synthase 1 Unknown Glutamate dehydrogenase 1, mitochondrial Glutaminase 2 Aminomethyltransferase, mitochondrial Dihydrolipoyl dehydrogenase Glycine cleavage system P protein L-Serine L-Histidine Pyruvic acid Carbamoyl phosphate Glycine L-Glutamic acid L-Glutamic acid L-Aspartic acid NH3 NH3 L-Asparagine H2O ATP L-Glutamine H2O AMP PPi Urocanic acid ATP CO2 H2O ADP Pi ATP ADP Pi L-Glutamine H2O NAD Oxoglutaric acid NADH H2O L-Glutamic acid Tetrahydrofolic acid 8-[(Aminomethyl)sulfanyl]-6- sulfanyloctanoic acid Dihydrolipoate 5,10-Methylene-THF NAD (R)-lipoic acid NADH CO2 Pyridoxal 5'-phosphate Biotin Magnesium FAD Pyridoxal 5'-phosphate Glycine and Serine Metabolism Histidine Metabolism Citric Acid Cycle Urea Cycle Glycine and Serine Metabolism Glutamate Metabolism Glutamate Metabolism Aspartate Metabolism Glycine cleavage system Mitochondrial Matrix Extracellular Space Intracellular Space Mitochondria
Mitochondria GCSH SDS ASRGL1 ASNS HAL CPS1 Unknown GLUD1 GLS2 AMT DLD GLDC L-Serine L-Histidine Pyruvic acid Carbamoyl phosphate Glycine L-Glutamic acid L-Glutamic acid L-Aspartic acid Ammonia Ammonia L-Asparagine Water Adenosine triphosphate L-Glutamine Water Adenosine monophosphate Pyrophosphate Urocanic acid Adenosine triphosphate Carbon dioxide Water Adenosine diphosphate Phosphoric acid Adenosine triphosphate Adenosine diphosphate Phosphate L-Glutamine Water NAD Oxoglutaric acid NADH Water L-Glutamic acid Tetrahydrofolic acid 8- [(Aminomethyl)sulfanyl]- 6- sulfanyloctanoic acid Dihydrolipoate 5,10- Methylene-THF NAD (R)-lipoic acid NADH Carbon dioxide Glycine and Serine Metabolism Histidine Metabolism Citric Acid Cycle Urea Cycle Glycine and Serine Metabolism Glutamate Metabolism Glutamate Metabolism Aspartate Metabolism
GCSH SDS ASRGL1 ASNS HAL CPS1 GLUD1 GLS2 AMT DLD GLDC Ser His Pyr CarbmP Gly Glu Glu Asp NH3 NH3 Asn H2O ATP Gln H2O AMP PPi Urocana ATP CO2 H2O ADP Pi ATP ADP Pi Gln H2O NAD AKG NADH H2O Glu THFA 8As6SA Dhlip 5XM-THF NAD Lipoate NADH CO2 Pyr-5'P Biotin Mg2+ FAD Pyr-5'P PW000157 PW000043 PW000005 PW000162 PW000157 PW000003 PW000003 PW000002 Glycine cleavage system Mitochondrial Matrix Extracellular Space Intracellular Space Mitochondria
Mitochondria GCSH SDS ASRGL1 ASNS HAL CPS1 GLUD1 GLS2 AMT DLD GLDC Ser His Pyr CarbmP Gly Glu Glu Asp NH3 NH3 Asn H2O ATP Gln H2O AMP Ppi Urocana ATP CO2 H2O ADP PhosfrA ATP ADP Pi Gln H2O NAD AKG NADH H2O Glu THFA 8As6SA Dhlip 5XM-THF NAD Lipoate NADH CO2 PW000157 PW000043 PW000005 PW000162 PW000157 PW000003 PW000003 PW000002