OTC - Ornithine carbamoyltransferase, mitochondrial - human protein (Function)
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OTC »  Ornithine carbamoyltransferase, mitochondrial   [ EC ]
Protein also known as:  Ornithine transcarbamylase (OTCase).
Gene name:  OTC
Family name: ATCase/OTCase
Entry whose protein(s) existence is based on evidence at protein level
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GO molecular function 
Amino acid bindingdefinition[GO:0016597] silver  
  • IEAOrtholog Compara
Phosphate ion bindingdefinition[GO:0042301] silver  
  • IEAOrtholog Compara
Phospholipid bindingdefinition[GO:0005543] silver  
  • IEAOrtholog Compara
GO biological process 
Anion homeostasisdefinition[GO:0055081] silver  
  • IEAOrtholog Compara
Arginine biosynthetic processdefinition[GO:0006526]  
  • IEAUniProtKB KW
Citrulline biosynthetic processdefinition[GO:0019240] silver  
  • IEAOrtholog Compara
Liver developmentdefinition[GO:0001889] silver  
  • IEAOrtholog Compara
Midgut developmentdefinition[GO:0007494] silver  
  • IEAOrtholog Compara
Protein homotrimerizationdefinition[GO:0070207] silver  
  • IEAOrtholog Compara
Response to biotindefinition[GO:0070781] silver  
  • IEAOrtholog Compara
Response to drugdefinition[GO:0042493] silver  
  • IEAOrtholog Compara
Response to insulindefinition[GO:0032868] silver  
  • IEAOrtholog Compara
Response to zinc iondefinition[GO:0010043] silver  
  • IEAOrtholog Compara
Urea cycledefinition[GO:0000050]  
  • IEAUniPathway
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC Carbamoyl phosphate + L-ornithine phosphate + L-citrulline.  
  • CuratedUniProtKB
It is regulated in the following manner
Negatively regulated by lysine acetylation.  
  • CuratedUniProtKB
This protein is involved in the following pathway
According to KEGG, this protein belongs to the following pathways:
Arginine and proline metabolism  hsa00330+5009  
Metabolic pathways  hsa01100+5009  
According to Reactome, this protein belongs to the following pathway:
Metabolism  REACT_111217  
More information is available from:


Biological process 
Amino-acid biosynthesis  definition   [KW-0028]
Arginine biosynthesis  definition   [KW-0055]
Urea cycle  definition   [KW-0835]
Molecular function 
Transferase  definition   [KW-0808]
Technical term 
Reference proteome  definition   [KW-1185]

Further external links

Enzyme and pathway databases
SABIO-RK: P00480
GeneWiki: Ornithine_transcarbamylase
GenomeRNAi: 5009
PRO: PR:P00480