Harmonizome 3.0

carbamoyl-phosphate synthetase 2, aspartate transcarbamylase, and dihydroorotase

The de novo synthesis of pyrimidine nucleotides is required for mammalian cells to proliferate. This gene encodes a trifunctional protein which is associated with the enzymatic activities of the first 3 enzymes in the 6-step pathway of pyrimidine biosynthesis: carbamoylphosphate synthetase (CPS II), aspartate transcarbamoylase, and dihydroorotase. This protein is regulated by the mitogen-activated protein kinase (MAPK) cascade, which indicates a direct link between activation of the MAPK cascade and de novo biosynthesis of pyrimidine nucleotides. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Apr 2015]

carbamoyl-phosphate synthase 1, mitochondrial

The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.[provided by RefSeq, May 2010]

From DrugBank Drug Targets

interacting proteins for the 4-AMINO-N-[(2-SULFANYLETHYL)CARBAMOYL]BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From Guide to Pharmacology Chemical Ligands of Receptors

receptors of the (3S)-3-{[(2S)-1-[(2S)-2-(2-aminoacetamido)-4-methylpentanoyl]pyrrolidin-2-yl]formamido}-3-({[(carboxymethyl)carbamoyl]methyl}carbamoyl)propanoic acid ligand (chemical) from the curated Guide to Pharmacology Chemical Ligands of Receptors dataset.

From CTD Gene-Chemical Interactions

genes/proteins interacting with the chemical N-(2-aminoethyl)-4-(5-(4-tolyl)-3-(trifluoromethyl)-1H-pyrazol-1-yl)benzenesulfonamide from the curated CTD Gene-Chemical Interactions dataset.

From CTD Gene-Chemical Interactions

genes/proteins interacting with the chemical 2,4-difluoro-N-(2-(methyloxy)-5-(4-(4-pyridazinyl)-6-quinolinyl)-3-pyridinyl)benzenesulfonamide from the curated CTD Gene-Chemical Interactions dataset.

From CTD Gene-Chemical Interactions

genes/proteins interacting with the chemical 4-(4-((glucopyranosyl)thiomethyl)-5-iodo-1-H-1,2,3-triazol-1-yl)benzenesulfonamide from the curated CTD Gene-Chemical Interactions dataset.

From CTD Gene-Chemical Interactions

genes/proteins interacting with the chemical 4-(5-(4-chlorophenyl)-3-(trifluoromethyl)-1H-pyrazol-1-yl)benzenesulfonamide from the curated CTD Gene-Chemical Interactions dataset.

From CTD Gene-Chemical Interactions

genes/proteins interacting with the chemical 3,N-dimethyl-N-(1-methyl-3-(4-methylpiperidin-1-yl)propyl)benzenesulfonamide from the curated CTD Gene-Chemical Interactions dataset.

From CTD Gene-Chemical Interactions

genes/proteins interacting with the chemical 4-(4-((2',3',4',6'-tetra-O-acetylglucopyranosyl)thiomethyl)-5-iodo-1-H-1,2,3-triazol-1-yl)benzenesulfonamide from the curated CTD Gene-Chemical Interactions dataset.

From DrugBank Drug Targets

interacting proteins for the 4-{[5-(CYCLOHEXYLOXY)[1,2,4]TRIAZOLO[1,5-A]PYRIMIDIN-7-YL]AMINO}BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the N-(2,2,2-TRIFLUOROETHYL)-N-{4-[2,2,2-TRIFLUORO-1-HYDROXY-1-(TRIFLUOROMETHYL)ETHYL]PHENYL}BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 4-(2-AMINOETHYL)BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 4-[(7-OXO-7H-THIAZOLO[5,4-E]INDOL-8-YLMETHYL)-AMINO]-N-PYRIDIN-2-YL-BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the N-(tert-butyl)-4-[5-(pyridin-2-ylamino)quinolin-3-yl]benzenesulfonamide drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the N-{3-METHYL-5-[2-(PYRIDIN-4-YLAMINO)-ETHOXY]-PHENYL}-BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the N-[(2-AMINO-6-METHYLPYRIMIDIN-4-YL)METHYL]-3-{[(E)-(2-OXODIHYDROFURAN-3(2H)-YLIDENE)METHYL]AMINO}BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 3-Nitro-4-(2-Oxo-Pyrrolidin-1-Yl)-Benzenesulfonamide drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the N-(CYCLOPROPYLMETHYL)-4-(METHYLOXY)-3-({5-[3-(3-PYRIDINYL)PHENYL]-1,3-OXAZOL-2-YL}AMINO)BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 2-chloro-5-[(1S)-1-hydroxy-3-oxo-2H-isoindol-1-yl]benzenesulfonamide drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 4-METHYL-N-METHYL-N-(2-PHENYL-2H-PYRAZOL-3-YL)BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 2,5-DICHLORO-N-[5-METHOXY-7-(6-METHOXYPYRIDIN-3-YL)-1,3-BENZOXAZOL-2-YL]BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 4-{5-[(Z)-(2-IMINO-4-OXO-1,3-THIAZOLIDIN-5-YLIDENE)METHYL]FURAN-2-YL}-2-(TRIFLUOROMETHYL)BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 4-{5-[(Z)-(2-IMINO-4-OXO-1,3-THIAZOLIDIN-5-YLIDENE)METHYL]FURAN-2-YL}BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 2,5-DICHLORO-N-(5-CHLORO-1,3-BENZOXAZOL-2-YL)BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 3-FLUORO-N-[1-(4-FLUOROPHENYL)-3-(2-THIENYL)-1H-PYRAZOL-5-YL]BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 3-(6-CYCLOHEXYLMETHOXY-9H-PURIN-2-YLAMINO)-BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 4-({[(4-METHYLPIPERAZIN-1-YL)AMINO]CARBONOTHIOYL}AMINO)BENZENESULFONAMIDE drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 3-({4-[(5-chloro-1,3-benzodioxol-4-yl)amino]pyrimidin-2-yl}amino)benzenesulfonamide drug from the curated DrugBank Drug Targets dataset.

From COMPARTMENTS Text-mining Protein Localization Evidence Scores

proteins co-occuring with the carbamoyl-phosphate synthase complex cellular component in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores dataset.

From CTD Gene-Chemical Interactions

genes/proteins interacting with the chemical 2-(((3,5-dichlorophenyl)carbamoyl)oxy)-2-methyl-3-butenoic acid from the curated CTD Gene-Chemical Interactions dataset.

From CTD Gene-Chemical Interactions

genes/proteins interacting with the chemical 1-(2-((1-ethoxycarbonyloxyethoxycarbonylmethyl)carbamoyl)-3-phenylpropyldisulfanylmethyl)-3-methylsulfanylpropylamine from the curated CTD Gene-Chemical Interactions dataset.

From CTD Gene-Chemical Interactions

genes/proteins interacting with the chemical 6-chloro-5-methyl-1-((2-(2-methylpyrid-3-yloxy)pyrid-5-yl)carbamoyl)indoline from the curated CTD Gene-Chemical Interactions dataset.

From CTD Gene-Chemical Interactions

genes/proteins interacting with the chemical (1'S, 2S)-4-amino-N-(1'-carbamoyl-2'-phenylethyl)-2-(4''-methyl-1''-naphthalenesulfonylamino)butanamide from the curated CTD Gene-Chemical Interactions dataset.

From CTD Gene-Disease Associations

genes/proteins associated with the disease Carbamoyl-Phosphate Synthase I Deficiency Disease from the curated CTD Gene-Disease Associations dataset.

From DISEASES Text-mining Gene-Disease Association Evidence Scores

genes co-occuring with the disease carbamoyl phosphate synthetase i deficiency disease in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores dataset.

From DrugBank Drug Targets

interacting proteins for the N-{1-[5-(1-Carbamoyl-2-Mercapto-Ethylcarbamoyl)-Pentylcarbamoyl]-2-[4-(Difluoro-Phosphono-Methyl)-Phenyl]-Ethyl}-3-{2-[4-(Difluoro-Phosphono-Methyl)-Phenyl]-Acetylamino}-Succinamic Acid drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 5-[(2-methyl-5-{[3-(trifluoromethyl)phenyl]carbamoyl}phenyl)amino]pyridine-3-carboxamide drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the N-({4-[(2-aminopyridin-4-yl)oxy]-3-fluorophenyl}carbamoyl)-2-(4-fluorophenyl)acetamide drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 2-(4-fluorophenyl)-N-{[3-fluoro-4-(1H-pyrrolo[2,3-b]pyridin-4-yloxy)phenyl]carbamoyl}acetamide drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 4-Benzoylamino-4-{1-{1-Carbamoyl-2-[4-(Difluoro-Phosphono-Methyl)-Phenyl]-Ethylcarbamoyl}-2-[4-(Difluoro-Phosphono-Methyl)-Phenyl]-Ethylcarbamoyl}-Butyric Acid drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 4-Carbamoyl-4-{[6-(Difluoro-Phosphono-Methyl)-Naphthalene-2-Carbonyl]-Amino}-Butyric Acid drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the {4-[2-Acetylamino-2-(3-Carbamoyl-2-Cyclohexylmethoxy-6,7,8,9-Tetrahydro-5h-Benzocyclohepten-5ylcarbamoyl)-Ethyl]-2-Phosphono-Phenyl}-Phosphonic Acid drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the N-Carbamoyl-Alanine drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 5-Chloro-1h-Indole-2-Carboxylic Acid{[Cyclopentyl-(2-Hydroxy-Ethyl)-Carbamoyl]-Methyl}-Amide drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the Cysteine-Methylene-Carbamoyl-1,10-Phenanthroline drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the N-({(1R)-1-carboxy-2-[(4-fluorobenzyl)sulfanyl]ethyl}carbamoyl)-L-glutamic acid drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the 1-Benzyloxycarbonylamino-2-Phenyl-Ethyl)-{2-[1-Carbamoyl-2-(1h-Indol-3-Yl)-Ethylcarbamoyl]-5-Phenyl-Pentyl}-Phosphinic Acid drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the trans-4-(7-carbamoyl-1H-benzimidazol-2-yl)-1-propylpiperidinium drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the (4-{2-Acetylamino-2-[1-(3-Carbamoyl-4-Cyclohexylmethoxy-Phenyl)-Ethylcarbamoyl}-Ethyl}-2-Phosphono-Phenoxy)-Acetic Acid drug from the curated DrugBank Drug Targets dataset.

From DrugBank Drug Targets

interacting proteins for the (2R)-2-(7-carbamoyl-1H-benzimidazol-2-yl)-2-methylpyrrolidinium drug from the curated DrugBank Drug Targets dataset.

From GeneRIF Biological Term Annotations

genes co-occuring with the biological term carbamoyl in literature-supported statements describing functions of genes from the GeneRIF Biological Term Annotations dataset.

From GO Biological Process Annotations 2015

genes participating in the carbamoyl phosphate metabolic process biological process from the curated GO Biological Process Annotations 2015 dataset.

From GO Biological Process Annotations 2015

genes participating in the carbamoyl phosphate biosynthetic process biological process from the curated GO Biological Process Annotations 2015 dataset.

From GO Molecular Function Annotations 2015

genes performing the carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity molecular function from the curated GO Molecular Function Annotations 2015 dataset.

From GO Molecular Function Annotations 2015

genes performing the carbamoyl-phosphate synthase (ammonia) activity molecular function from the curated GO Molecular Function Annotations 2015 dataset.

From Guide to Pharmacology Chemical Ligands of Receptors

receptors of the (3R 5R)-7-(5-{[(4-cyanophenyl)methyl]carbamoyl}-3-(4-fluorophenyl)-4-phenyl-1-(propan-2-yl)-1H-pyrrol-2-yl)-3 5-dihydroxyheptanoate ligand (chemical) from the curated Guide to Pharmacology Chemical Ligands of Receptors dataset.

From Guide to Pharmacology Chemical Ligands of Receptors

receptors of the (1S 2S 3S 5R)-1-(carboxymethyl)-3 5-bis({[(4-phenoxyphenyl)methyl](propyl)carbamoyl})cyclopentane-1 2-dicarboxylic acid ligand (chemical) from the curated Guide to Pharmacology Chemical Ligands of Receptors dataset.

From Guide to Pharmacology Chemical Ligands of Receptors

receptors of the (1S 2S 3S 4S)-3 4-bis[(2-methylpropyl)[(4-phenoxyphenyl)methyl]carbamoyl]cyclobutane-1 2-dicarboxylic acid ligand (chemical) from the curated Guide to Pharmacology Chemical Ligands of Receptors dataset.

From Guide to Pharmacology Chemical Ligands of Receptors

receptors of the (2S)-N-[(2S 3R)-1-cyclohexyl-4 4-difluoro-3-hydroxy-4-{[(2S)-2-methylbutyl]carbamoyl}butan-2-yl]-2-[(2S)-2-[(morpholine-4-sulfonyl)amino]-3-phenylpropanamido]pent-4-enamide ligand (chemical) from the curated Guide to Pharmacology Chemical Ligands of Receptors dataset.

From Guide to Pharmacology Chemical Ligands of Receptors

receptors of the (1S 2S 3S 4S)-3 4-bis({butyl[(4-phenoxyphenyl)methyl]carbamoyl})cyclobutane-1 2-dicarboxylic acid ligand (chemical) from the curated Guide to Pharmacology Chemical Ligands of Receptors dataset.

From Guide to Pharmacology Chemical Ligands of Receptors

receptors of the 2-({[(3-{[(2E)-3 7-dimethylocta-2 6-dien-1-yl]oxy}phenyl)methyl]carbamoyl}(hydroxy)amino)-2-methylpropanoate ligand (chemical) from the curated Guide to Pharmacology Chemical Ligands of Receptors dataset.

From Guide to Pharmacology Chemical Ligands of Receptors

receptors of the (2S 4R 5S 6S)-2-[(4S 5R)-4-(acetyloxy)-5-benzyl-3-methylidenehexyl]-2-carbamoyl-4-({[(2E 4S 6S)-4 6-dimethyloct-2-enoyl]oxy}methyl)-5-hydroxy-1 3-dioxane-4 5 6-tricarboxylic acid ligand (chemical) from the curated Guide to Pharmacology Chemical Ligands of Receptors dataset.

From Guide to Pharmacology Chemical Ligands of Receptors

receptors of the (6R 7R)-1-[(4S 5R)-4-(acetyloxy)-5-benzyl-3-methylidenehexyl]-4 7-dihydroxy-6-{[(11-phenoxyundecyl)carbamoyl]oxy}-2 8-dioxabicyclo[3.2.1]octane-3 4 5-tricarboxylic acid ligand (chemical) from the curated Guide to Pharmacology Chemical Ligands of Receptors dataset.

From Guide to Pharmacology Chemical Ligands of Receptors

receptors of the (1-methyl-1-{[3-(3-phenoxyphenyl)propyl]carbamoyl}ethyl)phosphonate ligand (chemical) from the curated Guide to Pharmacology Chemical Ligands of Receptors dataset.

From Guide to Pharmacology Chemical Ligands of Receptors

receptors of the (3R 5R)-7-[1-(4-fluorophenyl)-3-{methyl[(1R)-1-phenylethyl]carbamoyl}-4-(propan-2-yl)-1H-pyrazol-5-yl]-3 5-dihydroxyheptanoate ligand (chemical) from the curated Guide to Pharmacology Chemical Ligands of Receptors dataset.

From HMDB Metabolites of Enzymes

interacting proteins for the 3-Carbamoyl-2-phenylpropionaldehyde metabolite from the curated HMDB Metabolites of Enzymes dataset.

From HMDB Metabolites of Enzymes

interacting proteins for the Carbamoyl phosphate metabolite from the curated HMDB Metabolites of Enzymes dataset.

From HMDB Metabolites of Enzymes

interacting proteins for the 3-Carbamoyl-2-phenylpropionic acid metabolite from the curated HMDB Metabolites of Enzymes dataset.

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Carbamoyl-phosphate synthase, large subunit, N-terminal protein domain from the InterPro Predicted Protein Domain Annotations dataset.

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Aspartate/ornithine carbamoyltransferase, carbamoyl-P binding protein domain from the InterPro Predicted Protein Domain Annotations dataset.

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Carbamoyl-phosphate synthase, small subunit N-terminal domain protein domain from the InterPro Predicted Protein Domain Annotations dataset.

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domain protein domain from the InterPro Predicted Protein Domain Annotations dataset.

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Carbamoyl-phosphate synthase, large subunit protein domain from the InterPro Predicted Protein Domain Annotations dataset.

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Carbamoyl-phosphate synthetase, large subunit oligomerisation domain protein domain from the InterPro Predicted Protein Domain Annotations dataset.

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Carbamoyl-phosphate synthase large subunit, CPSase domain protein domain from the InterPro Predicted Protein Domain Annotations dataset.

From InterPro Predicted Protein Domain Annotations

proteins predicted to have the Carbamoyl-phosphate synthase, small subunit protein domain from the InterPro Predicted Protein Domain Annotations dataset.

From DisGeNET Gene-Disease Associations

genes associated with the disease Carbamoyl-Phosphate Synthase I Deficiency Disease in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.

From DisGeNET Gene-Disease Associations

genes associated with the disease Carbamoyl Phosphate Synthase 1 Deficiency in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.

From GO Biological Process Annotations 2023

genes participating in the carbamoyl phosphate biosynthetic process biological process from the curated GO Biological Process Annotations 2023 dataset.

From GO Biological Process Annotations 2023

genes participating in the carbamoyl phosphate metabolic process biological process from the curated GO Biological Process Annotations 2023 dataset.

From GO Molecular Function Annotations 2023

genes performing the carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity molecular function from the curated GO Molecular Function Annotations 2023 dataset.

From GO Molecular Function Annotations 2023

genes performing the carbamoyl-phosphate synthase (ammonia) activity molecular function from the curated GO Molecular Function Annotations 2023 dataset.

From GO Biological Process Annotations 2025

genes participating in the carbamoyl phosphate biosynthetic process biological process from the curated GO Biological Process Annotations 2025 dataset.

From GO Biological Process Annotations 2025

genes participating in the carbamoyl phosphate metabolic process biological process from the curated GO Biological Process Annotations 2025 dataset.

From GO Molecular Function Annotations 2025

genes performing the carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity molecular function from the curated GO Molecular Function Annotations 2025 dataset.

From GO Molecular Function Annotations 2025

genes performing the carbamoyl-phosphate synthase (ammonia) activity molecular function from the curated GO Molecular Function Annotations 2025 dataset.

From DISEASES Curated Gene-Disease Association Evidence Scores 2025

genes involed in the disease Carbamoyl phosphate synthetase I deficiency disease from the DISEASES Curated Gene-Disease Association Evidence Scores 2025 dataset.

From DISEASES Text-mining Gene-Disease Association Evidence Scores 2025

genes co-occuring with the disease Carbamoyl phosphate synthetase I deficiency disease in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores 2025 dataset.

From COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025

proteins co-occuring with the carbamoyl-phosphate synthase complex cellular component in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset.

From ClinVar Gene-Phenotype Associations 2025

genes associated with the CARBAMOYL PHOSPHATE SYNTHETASE I POLYMORPH phenotype from the curated ClinVar Gene-Phenotype Associations 2025 dataset.