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Urea transporter 2 CAD protein CAD protein CAD protein Dihydroorotate dehydrogenase (quinone), mitochondrial Uridine monophosphate synthetase isoform A Uridine monophosphate synthetase isoform A Soluble calcium- activated nucleotidase 1 UMP-CMP kinase 2, mitochondrial Nucleoside diphosphate kinase 6 Soluble calcium- activated nucleotidase 1 GTP:AMP phosphotransferase, mitochondrial Inosine triphosphate pyrophosphatase CTP synthase 1 Soluble calcium- activated nucleotidase 1 Nucleoside diphosphate kinase 6 Soluble calcium- activated nucleotidase 1 UMP-CMP kinase 2, mitochondrial Cytosolic purine 5'-nucleotidase Guanine deaminase Cytosolic purine 5'-nucleotidase Uridine phosphorylase 2 Dihydropyrimidine dehydrogenase [NADP(+)] Dihydropyrimidinase Beta- ureidopropionase Ribonucleoside- diphosphate reductase large subunit Nucleoside diphosphate kinase 6 UMP-CMP kinase 2, mitochondrial Cytosolic purine 5'-nucleotidase Uridine- cytidine kinase-like 1 Cytidine deaminase Thymidine phosphorylase Deoxycytidylate deaminase Thymidine kinase, cytosolic Thymidylate synthase Thymidylate synthase Soluble calcium- activated nucleotidase 1 Nucleoside diphosphate kinase 6 Soluble calcium- activated nucleotidase 1 Cytosolic purine 5'-nucleotidase Thymidine kinase, cytosolic Thymidine phosphorylase Dihydropyrimidine dehydrogenase [NADP(+)] Deoxyuridine 5'-triphosphate nucleotidohydrolase, mitochondrial Nucleoside diphosphate kinase 6 Thymidylate synthase Ribonucleoside- diphosphate reductase large subunit Uridine- cytidine kinase-like 1 Beta- ureidopropionase Dihydropyrimidinase Uridine triphosphate Cytidine triphosphate Uridine 5'-diphosphate CDP dCTP Thymidine 5'-triphosphate β-Alanine L-Glutamine Carbamoyl phosphate Phosphoribosyl pyrophosphate 3-Aminoisobutanoic acid Ureidoisobutyric acid Ureidoisobutyric acid ATP H2O HCO3- ADP Pi Ureidosuccinic acid L-Dihydroorotic acid Quinone Orotic acid Orotidylic acid Uridine 5'-monophosphate H2O Pi ATP ADP ADP ATP H2O Pi ADP AMP H2O PPi ATP ADP Pi dCTP H2O Pi ADP ATP H2O Cytidine monophosphate Pi ADP ATP H2O Cytidine Pi H2O Uridine NH3 H2O Pi Pi Uracil Ribose 1-phosphate NADP Dihydrouracil NADPH H2O Ureidopropionic acid H2O NH3 CO2 H2O dCDP ATP ADP dCMP ATP ADP H2O Deoxycytidine Pi ATP ADP H2O Deoxyuridine NH3 Pi Deoxyribose 1-phosphate H2O dUMP NH3 ATP ADP 5,10-Methylene-THF 5-Thymidylic acid Dihydrofolic acid ATP dTDP ADP H2O Pi ATP ADP H2O Pi H2O Thymidine Pi ATP ADP Pi Thymine Deoxyribose 1-phosphate NADP Dihydrothymine NADPH Deoxyuridine triphosphate H2O Pi ADP dUDP ATP ATP ADP Uridine 5'-diphosphate H2O ATP ADP H2O CO2 NH3 H2O Zinc (II) ion Zinc (II) ion Flavin Mononucleotide Calcium Magnesium Calcium Magnesium Calcium Magnesium Calcium Magnesium Magnesium FAD Flavin Mononucleotide Zinc (II) ion Magnesium FAD Calcium Magnesium Calcium FAD Flavin Mononucleotide Magnesium Fe2+ Zinc (II) ion quinol thioredoxin disulfide thioredoxin Thioredoxin disulfide Thioredoxin DNA reaction RNA reaction Alanine Metabolism Glutamate Metabolism Urea Cycle Pentose Phosphate Pathway Valine, Leucine, and Isoleucine Degradation Zinc (II) ion Zinc (II) ion Magnesium Magnesium Magnesium Zinc (II) ion Zinc (II) ion Fe2+ Zinc Intracellular Space Mitochondria Extracellular Space Symptoms include hypotonia, dystonic movements, scoliosis, microcephaly, and severe developmental delay.
SLC14A2 CAD CAD CAD DHODH UMPS UMPS CANT1 CMPK2 NME6 CANT1 AK3 ITPA CTPS1 CANT1 NME6 CANT1 CMPK2 NT5C2 GDA NT5C2 UPP2 DPYD DPYS UPB1 RRM2 RRM2B NME6 CMPK2 NT5C2 UCKL1 CDA TYMP DCTD TK1 TYMS TYMS CANT1 NME6 CANT1 NT5C2 TK1 TYMP DPYD DUT NME6 TYMS RRM2 RRM2B UCKL1 UPB1 DPYS Uridine triphosphate Cytidine triphosphate Uridine 5'-diphosphate CDP dCTP Thymidine 5'-triphosphate β-Alanine L-Glutamine Carbamoyl phosphate Phosphoribosyl pyrophosphate 3- Aminoisobutanoic acid Ureidoisobutyric acid Ureidoisobutyric acid Adenosine triphosphate Water Hydrogen carbonate Adenosine diphosphate Phosphate Ureidosuccinic acid L-Dihydroorotic acid Quinone Orotic acid Orotidylic acid Uridine 5'- monophosphate Water Phosphate Adenosine triphosphate Adenosine diphosphate Adenosine diphosphate Adenosine triphosphate Water Phosphoric acid Adenosine diphosphate 3'-AMP Water Pyrophosphate Adenosine triphosphate Adenosine diphosphate Phosphate dCTP Water Phosphate Adenosine diphosphate Adenosine triphosphate Water Cytidine monophosphate Phosphate Adenosine diphosphate Adenosine triphosphate Water Cytidine Phosphoric acid Water Uridine Ammonia Water Phosphate Phosphate Uracil Ribose 1-phosphate NADP Dihydrouracil NADPH Water Ureidopropionic acid Water Ammonia Carbon dioxide Water dCDP Adenosine triphosphate Adenosine diphosphate dCMP Adenosine triphosphate Adenosine diphosphate Water Deoxycytidine Phosphate Adenosine triphosphate Adenosine diphosphate Water Deoxyuridine Ammonia Phosphate Deoxyribose 1-phosphate Water dUMP Ammonia Adenosine triphosphate Adenosine diphosphate 5,10- Methylene-THF 5-Thymidylic acid Dihydrofolic acid Adenosine triphosphate dTDP Adenosine diphosphate Water Phosphate Adenosine triphosphate Adenosine diphosphate Water Phosphate Water Thymidine Phosphate Adenosine triphosphate Adenosine diphosphate Phosphate Thymine Deoxyribose 1-phosphate NADP Dihydrothymine NADPH Deoxyuridine triphosphate Water Phosphate Adenosine diphosphate dUDP Adenosine triphosphate Adenosine triphosphate Adenosine diphosphate Uridine 5'-diphosphate Water Adenosine triphosphate Adenosine diphosphate Water Carbon dioxide Ammonia Water quinol thioredoxin disulfide thioredoxin Thioredoxin disulfide Thioredoxin DNA reaction RNA reaction Alanine Metabolism Glutamate Metabolism Urea Cycle Pentose Phosphate Pathway Valine, Leucine, and Isoleucine Degradation
SLC14A2 CAD CAD CAD DHODH UMPS UMPS CANT1 CMPK2 NME6 CANT1 AK3 ITPA CTPS1 CANT1 NME6 CANT1 CMPK2 NT5C2 GDA NT5C2 UPP2 DPYD DPYS UPB1 RRM2 RRM2B NME6 CMPK2 NT5C2 UCKL1 CDA TYMP DCTD TK1 TYMS TYMS CANT1 NME6 CANT1 NT5C2 TK1 TYMP DPYD DUT NME6 TYMS RRM2 RRM2B UCKL1 UPB1 DPYS UTP CTP UDP CDP dCTP Tm5TP b-Ala Gln CarbmP PRPP AmnIsoB UrisoBa UrisoBa ATP H2O HCO3- ADP Pi USuccA L-Dhoa Quinone Orotate OMP U-5-P H2O Pi ATP ADP ADP ATP H2O Pi ADP AMP H2O PPi ATP ADP Pi dCTP H2O Pi ADP ATP H2O CMP Pi ADP ATP H2O Cytdin Pi H2O Uridine NH3 H2O Pi Pi Uracil Rib-1P NADP DUC NADPH H2O Ureidop H2O NH3 CO2 H2O dCDP ATP ADP dCMP ATP ADP H2O DC Pi ATP ADP H2O Du NH3 Pi D1P H2O dUMP NH3 ATP ADP 5XM-THF 5TMA FAH2 ATP dTDP ADP H2O Pi ATP ADP H2O Pi H2O T Pi ATP ADP Pi Thymine D1P NADP DHThy NADPH dUTP H2O Pi ADP dUDP ATP ATP ADP UDP H2O ATP ADP H2O CO2 NH3 H2O Zinc Zinc FlvnMnt Ca2+ Mg2+ Ca2+ Mg2+ Ca2+ Mg2+ Ca2+ Mg2+ Mg2+ FAD FlvnMnt Zinc Mg2+ FAD Ca2+ Mg2+ Ca2+ FAD FlvnMnt Mg2+ Fe2+ Zinc quinol TD Thiored TD Thiored DNA rea RNA rea PW000001 PW000003 PW000162 PW000055 PW000051 Zinc Zinc Mg2+ Mg2+ Mg2+ Zinc Zinc Fe2+ Zinc Intracellular Space Mitochondria Extracellular Space Symptoms include hypotonia, dystonic movements, scoliosis, microcephaly, and severe developmental delay.