Protein Name pyruvate carboxylase
Organism Homo sapiens
Gene ID 5091
Gene Symbol


UniProt P11498 (PYC_HUMAN), A0A024R5C5 (A0A024R5C5_HUMAN)
Relationships Total Number of functionally related compound(s) : 987
Total Number of Articles : 1653

pyruvate carboxylase

Gene Summary

This gene encodes pyruvate carboxylase, which requires biotin and ATP to catalyse the carboxylation of pyruvate to oxaloacetate. The active enzyme is a homotetramer arranged in a tetrahedron which is located exclusively in the mitochondrial matrix. Pyruvate carboxylase is involved in gluconeogenesis, lipogenesis, insulin secretion and synthesis of the neurotransmitter glutamate. Mutations in this gene have been associated with pyruvate carboxylase deficiency. Alternatively spliced transcript variants with different 5' UTRs, but encoding the same protein, have been found for this gene. [provided by RefSeq, Jul 2008]

  • pyruvate carboxylase, mitochondrial
  • pyruvic carboxylase
  • PCB
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