Version 5.4
Bos taurus Gene: PC
Summary
InnateDB Gene IDBG-644637.3
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol PC
Gene Name Pyruvate carboxylase, mitochondrial
Synonyms
Species Bos taurus
Ensembl Gene ENSBTAG00000019700
Encoded Proteins
IDBP-697292
Pyruvate carboxylase, mitochondrial
IDBP-697296
Pyruvate carboxylase, mitochondrial
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary This gene does not have any Entrez summary - the following is the summary from its human ortholog ENSG00000173599:
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]
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]
Gene Information
Type Protein coding
Genomic Location Chromosome 29:45508280-45611042
Strand Reverse strand
Band
Transcripts
ENSBTAT00000030039 ENSBTAP00000030027
ENSBTAT00000026258 ENSBTAP00000026258
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 0 experimentally validated interaction(s) in this database.
They are also associated with 13 interaction(s) predicted by orthology.
Predicted by orthology
Total 13 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0000166 nucleotide binding
GO:0003677 DNA binding
GO:0003824 catalytic activity
GO:0004075 biotin carboxylase activity
GO:0004736 pyruvate carboxylase activity
GO:0005515 protein binding
GO:0005524 ATP binding
GO:0008716 D-alanine-D-alanine ligase activity
GO:0016874 ligase activity
GO:0046872 metal ion binding
Biological Process
GO:0006094 gluconeogenesis
GO:0006629 lipid metabolic process
GO:0008152 metabolic process
Cellular Component
GO:0005739 mitochondrion
GO:0005743 mitochondrial inner membrane
GO:0005759 mitochondrial matrix
Orthologs
Species
Homo sapiens
Mus musculus
Gene ID
Gene Order
ENSG00000173599
View Gene Order
ENSMUSG00000024892
View Gene Order
Pathways
NETPATH
REACTOME
REACT_208874
Defective GIF causes intrinsic factor deficiency pathway
REACT_223924
Defects in cobalamin (B12) metabolism pathway
REACT_223300
Defective MUT causes methylmalonic aciduria mut type pathway
REACT_221274
Metabolism of carbohydrates pathway
REACT_218264
Defective MMAA causes methylmalonic aciduria type cblA pathway
REACT_227887
Defects in vitamin and cofactor metabolism pathway
REACT_217107
Defects in biotin (Btn) metabolism pathway
REACT_202103
Defective MMADHC causes methylmalonic aciduria and homocystinuria type cblD pathway
REACT_219973
Myoclonic epilepsy of Lafora pathway
REACT_210590
Defective TCN2 causes hereditary megaloblastic anemia pathway
REACT_224094
Defective HLCS causes multiple carboxylase deficiency pathway
REACT_221779
Defective MMACHC causes methylmalonic aciduria and homocystinuria type cblC pathway
REACT_210941
Defective BTD causes biotidinase deficiency pathway
REACT_222920
Defective MTRR causes methylmalonic aciduria and homocystinuria type cblE pathway
REACT_216256
Defective CD320 causes methylmalonic aciduria pathway
REACT_225666
Biotin transport and metabolism pathway
REACT_218209
Metabolism of water-soluble vitamins and cofactors pathway
REACT_209855
Metabolism of vitamins and cofactors pathway
REACT_227222
Gluconeogenesis pathway
REACT_211129
Glycogen storage diseases pathway
REACT_212778
Defective LMBRD1 causes methylmalonic aciduria and homocystinuria type cblF pathway
REACT_224672
Glucose metabolism pathway
REACT_219139
Metabolism pathway
REACT_225745
Defective MMAB causes methylmalonic aciduria type cblB pathway
REACT_223191
Defective CUBN causes hereditary megaloblastic anemia 1 pathway
REACT_207087
Disease pathway
REACT_212600
Defective AMN causes hereditary megaloblastic anemia 1 pathway
REACT_221291
Defective MTR causes methylmalonic aciduria and homocystinuria type cblG pathway
KEGG
INOH
PID NCI
Pathway Predictions based on Human Orthology Data
NETPATH
REACTOME
REACT_1520
Gluconeogenesis pathway
REACT_11153
Biotin transport and metabolism pathway
REACT_169312
Defective HLCS causes multiple carboxylase deficiency pathway
REACT_169313
Defective MUT causes methylmalonic aciduria mut type pathway
REACT_169316
Defective MMAA causes methylmalonic aciduria type cblA pathway
REACT_200783
Myoclonic epilepsy of Lafora pathway
REACT_169120
Defective TCN2 causes hereditary megaloblastic anemia pathway
REACT_11238
Metabolism of water-soluble vitamins and cofactors pathway
REACT_474
Metabolism of carbohydrates pathway
REACT_169280
Defective AMN causes hereditary megaloblastic anemia 1 pathway
REACT_169149
Defective MTR causes methylmalonic aciduria and homocystinuria type cblG pathway
REACT_169363
Defective LMBRD1 causes methylmalonic aciduria and homocystinuria type cblF pathway
REACT_169178
Defective CD320 causes methylmalonic aciduria pathway
REACT_169429
Defects in cobalamin (B12) metabolism pathway
REACT_169169
Defective MMACHC causes methylmalonic aciduria and homocystinuria type cblC pathway
REACT_169238
Defects in biotin (Btn) metabolism pathway
REACT_169403
Defective BTD causes biotidinase deficiency pathway
REACT_169415
Defective GIF causes intrinsic factor deficiency pathway
REACT_169385
Defects in vitamin and cofactor metabolism pathway
REACT_169132
Defective CUBN causes hereditary megaloblastic anemia 1 pathway
REACT_169256
Defective MMADHC causes methylmalonic aciduria and homocystinuria type cblD pathway
REACT_169439
Defective MTRR causes methylmalonic aciduria and homocystinuria type cblE pathway
REACT_111217
Metabolism pathway
REACT_169318
Defective MMAB causes methylmalonic aciduria type cblB pathway
REACT_116125
Disease pathway
REACT_723
Glucose metabolism pathway
REACT_200833
Glycogen storage diseases pathway
REACT_11193
Metabolism of vitamins and cofactors pathway
KEGG
hsa00620
Pyruvate metabolism pathway
hsa00020
Citrate cycle (TCA cycle) pathway
mmu00020
Citrate cycle (TCA cycle) pathway
mmu00620
Pyruvate metabolism pathway
INOH
INOH website
Citrate cycle pathway
INOH website
Glycolysis Gluconeogenesis pathway
INOH website
Alanine Aspartate Asparagine metabolism pathway
PID NCI
Cross-References
SwissProt
TrEMBL
UniProt Splice Variant
Entrez Gene
UniGene Bt.10147 Bt.97570
RefSeq NM_177946 XM_005226987 XM_005226988 XM_005226989 XM_005226990 XM_005226991 XM_005226992 XM_005226993 XM_005226994 XM_005226995
HUGO
OMIM
CCDS
HPRD
IMGT
EMBL
GenPept
RNA Seq Atlas

If you use InnateDB for your research, please cite the following publication:

Breuer et al., InnateDB: systems biology of innate immunity and beyond - recent updates and continuing curation. Nucl. Acids Res. (2013) 41 (D1) 

InnateDB is being developed jointly by the Brinkman Laboratory (Simon Fraser University, British Columbia, Canada), the Hancock Laboratory (University of British Columbia, Vancouver, British Columbia) and the Lynn EMBL Australia Group (South Australian Health & Medical Research Institute and Flinders University, Adelaide, Australia).

Funding is currently provided by Allergen and EMBL Australia. Previous funding has been provided by Genome Canada, the Foundation for the National Institutes of Health through the Grand Challenges in Global Health initiative and by Teagasc. InnateDB curated interactions are licensed under the Design Science License. All other data is licensed under the terms of the originating database. Contact: innatedb-mail@sfu.ca