pyruvate carboxylase

Symbol

PC (may also be known as: None)

Organism

Human

Description

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]

Orthologs

SymbolSpecies
pyc-1 Caenorhabditis elegans
CG1516 Drosophila melanogaster
pc Danio rerio
Pc Mus musculus
Pc Rattus norvegicus
PYC2 Saccharomyces cerevisiae
pyr1 Schizosaccharomyces pombe

Links to external resources

Changes associated with this gene

Identifier Name Type Tissues Organism Gene Data Actions
DAA3306 Gene expression of PC decreases with age Molecular skin Human PC —

GO Terms

GO IDGO TermGO Category
GO:0005975 carbohydrate metabolic process biological_process
GO:0006006 glucose metabolic process biological_process
GO:0006090 pyruvate metabolic process biological_process
GO:0006094 gluconeogenesis biological_process
GO:0006107 oxaloacetate metabolic process biological_process
GO:0008610 lipid biosynthetic process biological_process
GO:0044281 small molecule metabolic process biological_process
GO:0005625 soluble fraction cellular_component
GO:0005739 mitochondrion cellular_component
GO:0005743 mitochondrial inner membrane cellular_component
GO:0005759 mitochondrial matrix cellular_component
GO:0000166 nucleotide binding molecular_function
GO:0004075 biotin carboxylase activity molecular_function
GO:0004736 pyruvate carboxylase activity molecular_function
GO:0005524 ATP binding molecular_function
GO:0009374 biotin binding molecular_function
GO:0016874 ligase activity molecular_function
GO:0031406 carboxylic acid binding molecular_function
GO:0046872 metal ion binding molecular_function