EC: 6.3.4.16

carbamoyl-phosphate synthase (ammonia) (carbon-dioxide:ammonia ligase (ADP-forming, carbamate-phosphorylating))

enzyme lambda metabolic reaction experiment
- [N-Acetyl-L-glutamate]

2 ATP + Hydrogencarbonate + NH3 + L_Ornithine -> 2 ADP + 2 Phosphate + H2O + L_Citrulline		 (( (Vmax * S) ) / (Km + S)) buffer: 3.4 mM Hepes-KOH, 0.2 M Mannitol, 12.5 mM MgCl2, 8 mM Potassium phosphate, 0.7 mM EDTA, 1 mM Aminooxyacetate, 1 mM Succinate
PH: 7.4
Temperature: 24
- [N-Acetyl-L-glutamate]

Hydrogencarbonate + 2 ATP + L_Ornithine + NH3 -> L_Citrulline + H2O + 2 Phosphate + 2 ADP		 (( (Vmax * S) ) / (Km + S)) buffer: 3.4 mM Hepes-KOH, 0.2 M Mannitol, 12.5 mM MgCl2, 8 mM Potassium phosphate, 0.7 mM EDTA, 1 mM Aminooxyacetate, 1 mM Succinate
PH: 7.4
Temperature: 24
- [N-Acetyl-L-glutamate]

NH3 + 2 ATP + Hydrogencarbonate + L_Ornithine -> H2O + 2 ADP + 2 Phosphate + L_Citrulline		 (( (Vmax * S) ) / (Km + S)) buffer: 3.4 mM Hepes-KOH, 0.2 M Mannitol, 12.5 mM MgCl2, 8 mM Potassium phosphate, 0.7 mM EDTA, 1 mM Aminooxyacetate, 1 mM Succinate
PH: 7.4
Temperature: 24
- [N-Acetyl-L-glutamate]

Hydrogencarbonate + L_Ornithine + 2 ATP + NH3 -> L_Citrulline + 2 ADP + 2 Phosphate + H2O		 (( (Vmax * S) ) / (Km + S)) buffer: 3.4 mM Hepes-KOH, 0.2 M Mannitol, 12.5 mM MgCl2, 8 mM Potassium phosphate, 0.7 mM EDTA, 1 mM Aminooxyacetate, 1 mM Succinate
PH: 7.4
Temperature: 24
uniprot:P31327 [N-Acetyl glutamate]

H2O + 2 ATP + NH3 + CO2 -> 2 ADP + Carbamoyl_phosphate + Phosphate		 (( (( (kcat * E) ) * B) ) / (Km_B + B)) buffer: 50 mM Glycylglycine, 15 mM MgSO4, 5 mM L-Ornithine, 30 mM K+, 3 - 6 U/ml Carbamoyltransferase
PH: 7.6
Temperature: 37
uniprot:P31327 [N-Acetyl glutamate]

NH3 + H2O + 2 ATP + CO2 -> Phosphate + 2 ADP + Carbamoyl_phosphate		 (( (( (kcat * E) ) * A) ) / (Km_A + A)) buffer: 50 mM Glycylglycine, 15 mM MgSO4, 5 mM L-Ornithine, 30 mM K+, 3 - 6 U/ml Carbamoyltransferase
PH: 7.6
Temperature: 37
uniprot:P07756 [N-Acetyl glutamate]

H2O + 2 ATP + NH3 + CO2 -> Carbamoyl_phosphate + 2 ADP + Phosphate		 (( (( (kcat * E) ) * C) ) / (Km_C + C)) buffer: 50 mM Glycylglycine, 15 mM MgSO4, 5 mM L-Ornithine, 30 mM K+, 3 - 6 U/ml Carbamoyltransferase
PH: 7.6
Temperature: 37
uniprot:P31327 [N-Carbamyl-L-glutamate]

NH3 + 2 ATP + H2O + CO2 -> Carbamoyl_phosphate + 2 ADP + Phosphate		 (( (( (kcat * E) ) * D) ) / (Km_D + D)) buffer: 50 mM Glycylglycine, 15 mM MgSO4, 5 mM L-Ornithine, 30 mM K+, 3 - 6 U/ml Carbamoyltransferase
PH: 7.6
Temperature: 37
uniprot:P31327 [N-Acetyl glutamate]

NH3 + 2 ATP + CO2 + H2O -> Phosphate + 2 ADP + Carbamoyl_phosphate		 (( (( (kcat * E) ) * C) ) / (Km_C + C)) buffer: 50 mM Glycylglycine, 15 mM MgSO4, 5 mM L-Ornithine, 30 mM K+, 3 - 6 U/ml Carbamoyltransferase
PH: 7.6
Temperature: 37
uniprot:P31327 [N-Acetyl glutamate]

CO2 + H2O + NH3 + 2 ATP -> Phosphate + Carbamoyl_phosphate + 2 ADP		 (( (( (kcat * E) ) * D) ) / (Km_D + D)) buffer: 50 mM Glycylglycine, 15 mM MgSO4, 5 mM L-Ornithine, 30 mM K+, 3 - 6 U/ml Carbamoyltransferase
PH: 7.6
Temperature: 37

Pathways

pathway id name
BioCyc:HUMAN_PWY-4984 urea cycle
BioCyc:HUMAN_PWY-5686 UMP biosynthesis
BioCyc:META_PWY-5004 superpathway of L-citrulline metabolism
BioCyc:META_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:VCHO_PWY-4984 urea cycle
BioCyc:VCHO_PWY-7790 UMP biosynthesis II
BioCyc:VCHO_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
BioCyc:MTBH37RV_GLUTAMINDEG-PWY L-glutamine degradation I
BioCyc:MTBCDC1551_GLUTAMINDEG-PWY L-glutamine degradation I
BioCyc:MTBCDC1551_PWY-7400 L-arginine biosynthesis IV (archaebacteria)
BioCyc:ANTHRA_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
BioCyc:ANTHRA_PWY-4984 urea cycle
BioCyc:AGRO_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:MOUSE_PWY-4984 urea cycle
BioCyc:MOUSE_PWY0-162 pyrimidine ribonucleotides de novo biosynthesis
BioCyc:BSUB_PWY-7791 UMP biosynthesis III
BioCyc:ECOL316407_ARG+POLYAMINE-SYN superpathway of arginine and polyamine biosynthesis
BioCyc:CORYNE_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:GCF_000013425_PWY-5686 UMP biosynthesis I
BioCyc:HUMAN_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:HUMAN_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:META_PWY-4984 urea cycle
BioCyc:META_PWY-5686 UMP biosynthesis I
BioCyc:ECO_ARG+POLYAMINE-SYN superpathway of arginine and polyamine biosynthesis
BioCyc:ECO_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
BioCyc:ECO_PWY-5686 UMP biosynthesis I
BioCyc:ARA_PWY-5686 UMP biosynthesis I
BioCyc:ARA_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:ARA_PWY-4984 urea cycle
BioCyc:ARA_PWY-7060 ornithine-citrulline shuttle
BioCyc:MTBH37RV_PWY-5686 UMP biosynthesis I
BioCyc:MTBH37RV_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:MTBH37RV_ARGSYNBSUB-PWY L-arginine biosynthesis II (acetyl cycle)
BioCyc:MTBH37RV_CITRULLINE-DEG-PWY L-citrulline degradation
BioCyc:MTBCDC1551_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
BioCyc:MTBCDC1551_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:ECOO157_GLUTAMINDEG-PWY glutamine degradation I
BioCyc:ECOO157_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:ECOO157_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:ECOO157_PWY-7400 arginine biosynthesis IV (archaebacteria)
BioCyc:ECOL199310_PWY-5686 UMP biosynthesis
BioCyc:ECOL199310_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:ECOL199310_PWY-7400 arginine biosynthesis IV (archaebacteria)
BioCyc:ECOL199310_ARGSYN-PWY arginine biosynthesis I (via L-ornithine)
BioCyc:ANTHRA_PWY-5686-1 UMP biosynthesis
BioCyc:ANTHRA_ARGSYNBSUB-PWY L-arginine biosynthesis II (acetyl cycle)
BioCyc:AGRO_PWY-5686 UMP biosynthesis I
BioCyc:AGRO_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
BioCyc:MOUSE_PWY-5004 superpathway of citrulline metabolism
BioCyc:ECOL316407_PWY-5686 UMP biosynthesis
BioCyc:CORYNE_ARGSYNBSUB-PWY L-arginine biosynthesis II (acetyl cycle)
BioCyc:CORYNE_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:META_PWY-7790 UMP biosynthesis II
BioCyc:META_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
BioCyc:META_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:ECO_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:YEAST_PWY-4984 urea cycle
BioCyc:YEAST_PWY-7790 UMP biosynthesis II
BioCyc:ARA_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:ARA_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
BioCyc:VCHO_GLUTAMINDEG-PWY L-glutamine degradation I
BioCyc:MTBH37RV_ARGDEGRAD-PWY L-arginine degradation V (arginine deiminase pathway)
BioCyc:MTBH37RV_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:MTBCDC1551_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:MTBCDC1551_PWY-5686 UMP biosynthesis
BioCyc:ECOL199310_GLUTAMINDEG-PWY glutamine degradation I
BioCyc:ECOL199310_ARGSYNBSUB-PWY arginine biosynthesis II (acetyl cycle)
BioCyc:CAULO_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:CAULO_ARGSYNBSUB-PWY arginine biosynthesis II (acetyl cycle)
BioCyc:CAULO_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:AGRO_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:MOUSE_PWY-5686 uridine-5'-phosphate biosynthesis
BioCyc:BSUB_PWY-5686-1 uridine-5'-phosphate biosynthesis
BioCyc:ECOL316407_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:ECOL316407_ARGSYN-PWY arginine biosynthesis I (via L-ornithine)
BioCyc:CORYNE_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
BioCyc:CORYNE_GLUTAMINDEG-PWY L-glutamine degradation I
BioCyc:GCF_000013425_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:GCF_000013425_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:META_PWY-7791 UMP biosynthesis III
BioCyc:META_ARG+POLYAMINE-SYN superpathway of arginine and polyamine biosynthesis
BioCyc:ECO_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:YEAST_PYRIMID-RNTSYN-PWY de novo biosynthesis of pyrimidine ribonucleotides
BioCyc:YEAST_ARGSYNBSUB-PWY arginine biosynthesis
BioCyc:ARA_ARGSYNBSUB-PWY L-arginine biosynthesis II (acetyl cycle)
BioCyc:VCHO_ARGSYNBSUB-PWY L-arginine biosynthesis II (acetyl cycle)
BioCyc:MTBH37RV_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
BioCyc:MTBCDC1551_ARGSYNBSUB-PWY L-arginine biosynthesis II (acetyl cycle)
BioCyc:ECOO157_PWY-5686 UMP biosynthesis
BioCyc:ECOO157_ARGSYN-PWY arginine biosynthesis I (via L-ornithine)
BioCyc:ECOL199310_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:CAULO_ARGSYN-PWY arginine biosynthesis I
BioCyc:CAULO_PWY-5686 UMP biosynthesis
BioCyc:CAULO_GLUTAMINDEG-PWY glutamine degradation I
BioCyc:ANTHRA_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
BioCyc:ANTHRA_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
BioCyc:AGRO_PWY-4984 urea cycle
BioCyc:BSUB_ARGSYNBSUB-PWY L-arginine biosynthesis II (acetyl cycle)
BioCyc:CORYNE_PWY-5686 UMP biosynthesis
BioCyc:GCF_000013425_ARGSYNBSUB-PWY L-arginine biosynthesis II (acetyl cycle)
WikiPathways:WP2472 Escherichia coli K-12 peripherome
PathBank:SMP0000812 Arginine Metabolism
PathBank:SMP0000959 Pyrimidine Metabolism
PathBank:SMP0001888 Operon: Carbamoyl Phosphate Synthetase
PlantCyc:ARA_PWY-7211 superpathway of pyrimidine deoxyribonucleotides de novo biosynthesis
PlantCyc:ARA_PWY-7060 ornithine-citrulline shuttle
PlantCyc:ARA_PWY-5686 UMP biosynthesis I
PlantCyc:ARA_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
PlantCyc:ARA_PWY-4984 urea cycle
PlantCyc:ARA_ARGSYNBSUB-PWY L-arginine biosynthesis II (acetyl cycle)
PlantCyc:ARA_PWY-5004 superpathway of L-citrulline metabolism
PlantCyc:ARA_PWY0-162 superpathway of pyrimidine ribonucleotides de novo biosynthesis
Reactome:R-CEL-500753 Pyrimidine biosynthesis
Reactome:R-DDI-8956320 Nucleotide biosynthesis
Reactome:R-DME-500753 Pyrimidine biosynthesis
Reactome:R-MMU-1430728 Metabolism
Reactome:R-SCE-500753 Pyrimidine biosynthesis
Reactome:R-SCE-71291 Amino acid and derivative metabolism
Reactome:R-SPO-1430728 Metabolism
Reactome:R-SPO-15869 Nucleotide metabolism
Reactome:R-DDI-15869 Nucleotide metabolism
Reactome:R-DME-1430728 Metabolism
Reactome:R-HSA-8956320 Nucleotide biosynthesis
Reactome:R-MMU-8956320 Nucleotide biosynthesis
Reactome:R-SCE-1430728 Metabolism
Reactome:R-SPO-500753 Pyrimidine biosynthesis
Reactome:R-SPO-71291 Amino acid and derivative metabolism
Reactome:R-SPO-70635 Urea cycle
Reactome:R-CEL-15869 Nucleotide metabolism
Reactome:R-DME-15869 Nucleotide metabolism
Reactome:R-HSA-500753 Pyrimidine biosynthesis
Reactome:R-HSA-71291 Amino acid and derivative metabolism
Reactome:R-MMU-500753 Pyrimidine biosynthesis
Reactome:R-MMU-71291 Amino acid and derivative metabolism
Reactome:R-RNO-1430728 Metabolism
Reactome:R-RNO-71291 Amino acid and derivative metabolism
Reactome:R-SCE-15869 Nucleotide metabolism
Reactome:R-SPO-8956320 Nucleotide biosynthesis
Reactome:R-CEL-1430728 Metabolism
Reactome:R-CEL-8956320 Nucleotide biosynthesis
Reactome:R-DDI-1430728 Metabolism
Reactome:R-DDI-500753 Pyrimidine biosynthesis
Reactome:R-DME-8956320 Nucleotide biosynthesis
Reactome:R-HSA-1430728 Metabolism
Reactome:R-HSA-15869 Nucleotide metabolism
Reactome:R-HSA-70635 Urea cycle
Reactome:R-MMU-15869 Nucleotide metabolism
Reactome:R-MMU-70635 Urea cycle
Reactome:R-RNO-70635 Urea cycle
Reactome:R-SCE-8956320 Nucleotide biosynthesis
Reactome:R-SCE-70635 Urea cycle
WikiPathways:WP4595 Urea cycle and associated pathways
WikiPathways:WP4225 Pyrimidine metabolism and related diseases
WikiPathways:WP497 Urea cycle and metabolism of amino groups
PathBank:SMP0120666 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0120678 beta-Ureidopropionase Deficiency
PathBank:SMP0120682 Carbamoyl Phosphate Synthetase Deficiency
PathBank:SMP0120686 Citrullinemia Type I
PathBank:SMP0120710 Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)
PathBank:SMP0120714 Homocarnosinosis
PathBank:SMP0120722 Hypoacetylaspartia
PathBank:SMP0120750 MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy)
PathBank:SMP0120758 Ornithine Aminotransferase Deficiency (OAT Deficiency)
PathBank:SMP0120762 Ornithine Transcarbamylase Deficiency (OTC Deficiency)
PathBank:SMP0120790 Hyperornithinemia-Hyperammonemia-Homocitrullinuria [HHH-syndrome]
PathBank:SMP0120850 Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0108157 Apoptotic DNA Fragmentation and Tissue Homeostasis
PathBank:SMP0120456 Argininosuccinic Aciduria
PathBank:SMP0120488 Glycogen Storage Disease Type 1A (GSD1A) or Von Gierke Disease
PathBank:SMP0120496 Hyperinsulinism-Hyperammonemia Syndrome
PathBank:SMP0120504 Lactic Acidemia
PathBank:SMP0120569 Creatine Deficiency, Guanidinoacetate Methyltransferase Deficiency
PathBank:SMP0120613 2-Ketoglutarate Dehydrogenase Complex Deficiency
PathBank:SMP0120637 Glycogenosis, Type IB
PathBank:SMP0120645 Glycogenosis, Type IA. Von Gierke Disease
PathBank:SMP0000059 Urea Cycle
PathBank:SMP0000020 Arginine and Proline Metabolism
PathBank:SMP0000009 Ammonia Recycling
PathBank:SMP0000339 Hyperinsulinism-Hyperammonemia Syndrome
PathBank:SMP0000362 Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)
PathBank:SMP0000188 Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)
PathBank:SMP0000001 Citrullinemia Type I
PathBank:SMP0000178 Dihydropyrimidinase Deficiency
PathBank:SMP0000504 Creatine Deficiency, Guanidinoacetate Methyltransferase Deficiency
PathBank:SMP0002420 Proline Metabolism
PathBank:SMP0087278 Glutamate Metabolism
PathBank:SMP0087282 Ammonia Recycling
PathBank:SMP0087426 Aspartate Metabolism
PathBank:SMP0063579 Ammonia Recycling
PathBank:SMP0063596 Arginine and Proline Metabolism
PathBank:SMP0063661 Pyruvate Metabolism
PathBank:SMP0120677 Argininosuccinic Aciduria
PathBank:SMP0120681 Canavan Disease
PathBank:SMP0120693 Dihydropyrimidinase Deficiency
PathBank:SMP0120721 Hyperprolinemia Type II
PathBank:SMP0120761 Prolidase Deficiency (PD)
PathBank:SMP0120789 Hyperornithinemia with Gyrate Atrophy (HOGA)
PathBank:SMP0108760 Glutaminolysis and Cancer
PathBank:SMP0120455 Argininemia
PathBank:SMP0120499 Hyperprolinemia Type I
PathBank:SMP0120507 UMP Synthase Deficiency (Orotic Aciduria)
PathBank:SMP0120531 Pyruvate Dehydrogenase Complex Deficiency
PathBank:SMP0120535 Pyruvate Carboxylase Deficiency
PathBank:SMP0120539 Prolinemia Type II
PathBank:SMP0120543 Primary Hyperoxaluria Type I
PathBank:SMP0120572 L-Arginine:Glycine Amidinotransferase Deficiency
PathBank:SMP0120612 Mitochondrial Complex II Deficiency
PathBank:SMP0120624 Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1)
PathBank:SMP0000046 Pyrimidine Metabolism
PathBank:SMP0000385 Homocarnosinosis
PathBank:SMP0000207 Prolidase Deficiency (PD)
PathBank:SMP0000208 Prolinemia Type II
PathBank:SMP0000003 Argininosuccinic Aciduria
PathBank:SMP0000219 UMP Synthase Deficiency (Orotic Aciduria)
PathBank:SMP0000136 2-Hydroxyglutric Aciduria (D and L Form)
PathBank:SMP0000507 L-Arginine:Glycine Amidinotransferase Deficiency
PathBank:SMP0000567 Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0002416 Arginine Metabolism
PathBank:SMP0087473 Pyrimidine Metabolism
PathBank:SMP0063617 Gluconeogenesis
PathBank:SMP0063772 Apoptotic DNA Fragmentation and Tissue Homeostasis
PathBank:SMP0120676 Argininemia
PathBank:SMP0120716 Hyperinsulinism-Hyperammonemia Syndrome
PathBank:SMP0120788 Creatine Deficiency, Guanidinoacetate Methyltransferase Deficiency
PathBank:SMP0108234 Apoptotic DNA Fragmentation and Tissue Homeostasis
PathBank:SMP0108758 The Oncogenic Action of 2-Hydroxyglutarate
PathBank:SMP0120445 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0120461 Canavan Disease
PathBank:SMP0120473 Dihydropyrimidinase Deficiency
PathBank:SMP0120501 Hyperprolinemia Type II
PathBank:SMP0120533 Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency)
PathBank:SMP0120541 Prolidase Deficiency (PD)
PathBank:SMP0120570 Hyperornithinemia with Gyrate Atrophy (HOGA)
PathBank:SMP0120610 Congenital Lactic Acidosis
PathBank:SMP0120614 Pyruvate Dehydrogenase Deficiency (E3)
PathBank:SMP0120622 Primary Hyperoxaluria II, PH2
PathBank:SMP0120626 Fructose-1,6-diphosphatase Deficiency
PathBank:SMP0120638 Glycogenosis, Type IC
PathBank:SMP0000067 Aspartate Metabolism
PathBank:SMP0000360 Hyperprolinemia Type II
PathBank:SMP0000363 Ornithine Aminotransferase Deficiency (OAT Deficiency)
PathBank:SMP0000175 Canavan Disease
PathBank:SMP0000205 Ornithine Transcarbamylase Deficiency (OTC Deficiency)
PathBank:SMP0000202 MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy)
PathBank:SMP0000505 Hyperornithinemia with Gyrate Atrophy (HOGA)
PathBank:SMP0002370 Pyrimidine Metabolism
PathBank:SMP0087276 Aspartate Metabolism
PathBank:SMP0087340 Pyrimidine Metabolism
PathBank:SMP0087428 Glutamate Metabolism
PathBank:SMP0063586 Alanine Metabolism
PathBank:SMP0063590 Glutamate Metabolism
PathBank:SMP0063658 Pyrimidine Metabolism
PathBank:SMP0120659 2-Hydroxyglutric Aciduria (D and L Form)
PathBank:SMP0120675 Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)
PathBank:SMP0120719 Hyperprolinemia Type I
PathBank:SMP0120727 UMP Synthase Deficiency (Orotic Aciduria)
PathBank:SMP0120759 Prolinemia Type II
PathBank:SMP0120791 L-Arginine:Glycine Amidinotransferase Deficiency
PathBank:SMP0120438 2-Hydroxyglutric Aciduria (D and L Form)
PathBank:SMP0120454 Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)
PathBank:SMP0120458 beta-Ureidopropionase Deficiency
PathBank:SMP0120462 Carbamoyl Phosphate Synthetase Deficiency
PathBank:SMP0120466 Citrullinemia Type I
PathBank:SMP0120490 Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)
PathBank:SMP0120494 Homocarnosinosis
PathBank:SMP0120502 Hypoacetylaspartia
PathBank:SMP0120506 Leigh Syndrome
PathBank:SMP0120530 MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy)
PathBank:SMP0120538 Ornithine Aminotransferase Deficiency (OAT Deficiency)
PathBank:SMP0120542 Ornithine Transcarbamylase Deficiency (OTC Deficiency)
PathBank:SMP0120571 Hyperornithinemia-Hyperammonemia-Homocitrullinuria [HHH-syndrome]
PathBank:SMP0120611 Fumarase Deficiency
PathBank:SMP0120615 Pyruvate Dehydrogenase Deficiency (E2)
PathBank:SMP0120623 Pyruvate Kinase Deficiency
PathBank:SMP0120627 Triosephosphate Isomerase Deficiency
PathBank:SMP0120631 Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0000072 Glutamate Metabolism
PathBank:SMP0000243 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0000361 Hyperprolinemia Type I
PathBank:SMP0000192 Hypoacetylaspartia
PathBank:SMP0000357 Argininemia
PathBank:SMP0000172 beta-Ureidopropionase Deficiency
PathBank:SMP0000002 Carbamoyl Phosphate Synthetase Deficiency
PathBank:SMP0000506 Hyperornithinemia-Hyperammonemia-Homocitrullinuria [HHH-syndrome]
PathBank:SMP0086930 Warburg Effect
PathBank:SMP0087283 Arginine and Proline Metabolism
PathBank:SMP0087319 Urea Cycle
PathBank:SMP0087431 Ammonia Recycling
PathBank:SMP0063477 Citric Acid Cycle
PathBank:SMP0063597 Aspartate Metabolism
PathBank:SMP0063681 Transfer of Acetyl Groups into Mitochondria
PathBank:SMP0063686 Urea Cycle