PC - Pyruvate carboxylase, mitochondrial - human protein (Function)
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PC »  Pyruvate carboxylase, mitochondrial   [ EC ]
Protein also known as:  Pyruvic carboxylase (PCB).
Gene name:  PC
Entry whose protein(s) existence is based on evidence at protein level
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Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate.  
  • CuratedUniProtKB
GO molecular function 
ATP bindingdefinition[GO:0005524] silver  
  • IEAOrtholog Compara
Biotin bindingdefinition[GO:0009374] silver  
  • IEAOrtholog Compara
Biotin carboxylase activitydefinition[GO:0004075] silver  
  • IEAInterPro 2 GO
DNA bindingdefinition[GO:0003677] silver  
  • IEAInterPro 2 GO
Metal ion bindingdefinition[GO:0046872]  
  • IEAUniProtKB KW
Protein bindingdefinition[GO:0005515]  
  • IPIIntAct
GO biological process 
  • IEAUniPathway
Lipid metabolic processdefinition[GO:0006629]  
  • IEAUniProtKB KW
Oxaloacetate metabolic processdefinition[GO:0006107] silver  
  • IEAOrtholog Compara
Pyruvate metabolic processdefinition[GO:0006090] silver  
  • IEAOrtholog Compara
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC ATP + pyruvate + HCO(3)(-) ADP + phosphate + oxaloacetate.  
  • CuratedUniProtKB
It requires the following cofactors
  • CuratedUniProtKB
Binds 1 manganese ion per subunit.  
  • CuratedUniProtKB
More information is available from:
This protein is involved in the following pathway
According to KEGG, this protein belongs to the following pathways:
Citrate cycle (TCA cycle)  hsa00020+5091  
Metabolic pathways  hsa01100+5091  
Pyruvate metabolism  hsa00620+5091  
According to Reactome, this protein belongs to the following pathways:
Biotin transport and metabolism  REACT_11153  
Defective HLCS causes multiple carboxylase deficiency  REACT_169312  
Gluconeogenesis  REACT_1520  
More information is available from:


Biological process 
Gluconeogenesis  definition   [KW-0312]
Lipid biosynthesis  definition   [KW-0444]
Lipid metabolism  definition   [KW-0443]
Molecular function 
Ligase  definition   [KW-0436]
Technical term 
Multifunctional enzyme  definition   [KW-0511]
Reference proteome  definition   [KW-1185]

Further external links

Enzyme and pathway databases
SABIO-RK: P11498
GenomeRNAi: 5091
PRO: PR:P11498