CPS1 - Carbamoyl-phosphate synthase [ammonia], mitochondrial - human protein (Function)
 
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CPS1 »  Carbamoyl-phosphate synthase [ammonia], mitochondrial   [ EC 6.3.4.16 ]
 
Protein also known as:  Carbamoyl-phosphate synthetase I (CPSase I).
Gene name:  CPS1
Entry whose protein(s) existence is based on evidence at protein level
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1 69 3
GENE REF ISO

Function

 show evidences
Overview 
Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.  
  • CuratedUniProtKB
GO molecular function 
ATP bindingdefinition[GO:0005524] silver  
  • IEAOrtholog Compara
Calcium ion bindingdefinition[GO:0005509] silver  
  • IEAOrtholog Compara
Carbamoyl-phosphate synthase (ammonia) activitydefinition[GO:0004087]  
2
  • IMPBHF-UCL
Endopeptidase activitydefinition[GO:0004175] silver  
  • IEAOrtholog Compara
Glutamate bindingdefinition[GO:0016595] silver  
  • IEAOrtholog Compara
Modified amino acid bindingdefinition[GO:0072341]  
1
  • IDAUniProtKB
Phospholipid bindingdefinition[GO:0005543] silver  
  • IEAOrtholog Compara
Protein bindingdefinition[GO:0005515]  
3
  • IPIIntAct
Protein complex bindingdefinition[GO:0032403] silver  
  • IEAOrtholog Compara
GO biological process 
Anion homeostasisdefinition[GO:0055081] silver  
  • IEAOrtholog Compara
Carbamoyl phosphate biosynthetic processdefinition[GO:0070409]  
1
  • IMPUniProtKB
Cellular response to cAMPdefinition[GO:0071320] silver  
  • IEAOrtholog Compara
Cellular response to fibroblast growth factor stimulusdefinition[GO:0044344] silver  
  • IEAOrtholog Compara
Cellular response to glucagon stimulusdefinition[GO:0071377] silver  
  • IEAOrtholog Compara
Cellular response to oleic aciddefinition[GO:0071400] silver  
  • IEAOrtholog Compara
Citrulline biosynthetic processdefinition[GO:0019240]  
1
  • NASBHF-UCL
Glutamine catabolic processdefinition[GO:0006543] silver  
  • IEAInterPro 2 GO
Glycogen catabolic processdefinition[GO:0005980]  
1
  • IMPBHF-UCL
Hepatocyte differentiationdefinition[GO:0070365] silver  
  • IEAOrtholog Compara
Homocysteine metabolic processdefinition[GO:0050667]  
1
  • IDAUniProtKB
Midgut developmentdefinition[GO:0007494] silver  
  • IEAOrtholog Compara
Nitric oxide metabolic processdefinition[GO:0046209]  
1
  • IMPBHF-UCL
Positive regulation of vasodilationdefinition[GO:0045909]  
1
  • IMPBHF-UCL
Proteolysisdefinition[GO:0006508]  
  • IEAOrtholog Compara
Response to aminedefinition[GO:0014075] silver  
  • IEAOrtholog Compara
Response to amino aciddefinition[GO:0043200] silver  
  • IEAOrtholog Compara
Response to dexamethasonedefinition[GO:0071548] silver  
  • IEAOrtholog Compara
Response to drugdefinition[GO:0042493] silver  
  • IEAOrtholog Compara
Response to fooddefinition[GO:0032094] silver  
  • IEAOrtholog Compara
Response to growth hormonedefinition[GO:0060416] silver  
  • IEAOrtholog Compara
Response to lipopolysaccharidedefinition[GO:0032496]  
1
  • IDAUniProtKB
Response to starvationdefinition[GO:0042594] silver  
  • IEAOrtholog Compara
Response to toxic substancedefinition[GO:0009636] silver  
  • IEAOrtholog Compara
Response to zinc iondefinition[GO:0010043] silver  
  • IEAOrtholog Compara
Triglyceride catabolic processdefinition[GO:0019433]  
1
  • IMPBHF-UCL
Urea cycledefinition[GO:0000050]  
1
  • NASBHF-UCL
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 6.3.4.16: 2 ATP + NH(3) + CO(2) + H(2)O 2 ADP + phosphate + carbamoyl phosphate.  
  • CuratedUniProtKB
It is regulated in the following manner
Requires N-acetyl-L-glutamate (NAG) as an allosteric activator.  
  • CuratedUniProtKB
 
More information is available from:
Pathways 
According to KEGG, this protein belongs to the following pathways:
Alanine, aspartate and glutamate metabolism  hsa00250+1373  
Arginine and proline metabolism  hsa00330+1373  
Metabolic pathways  hsa01100+1373  
Nitrogen metabolism  hsa00910+1373  
According to Reactome, this protein belongs to the following pathway:
Urea cycle  REACT_847  
 
More information is available from:
 

Keywords

Biological process 
Urea cycle  definition   [KW-0835]
Molecular function 
Ligase  definition   [KW-0436]
Technical term 
Allosteric enzyme  definition   [KW-0021]
Reference proteome  definition   [KW-1185]
 

Further external links

Enzyme and pathway databases
SABIO-RK: P31327
Other
GenomeRNAi: 1373
PRO: PR:P31327