Bos taurus Gene: BT.55189
Summary
InnateDB Gene IDBG-633259.3
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol BT.55189
Gene Name propionyl-CoA carboxylase alpha chain, mitochondrial
Synonyms
Species Bos taurus
Ensembl Gene ENSBTAG00000010672
Encoded Proteins
propionyl-CoA carboxylase alpha chain, 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 ENSG00000175198:
The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010]
Gene Information
Type Protein coding
Genomic Location Chromosome 12:80793559-81155392
Strand Forward strand
Band
Transcripts
ENSBTAT00000014120 ENSBTAP00000014120
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 2 interaction(s) predicted by orthology.
Predicted by orthology
Total 2 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0000166 nucleotide binding
GO:0003824 catalytic activity
GO:0004075 biotin carboxylase activity
GO:0004658 propionyl-CoA carboxylase activity
GO:0005524 ATP binding
GO:0008716 D-alanine-D-alanine ligase activity
GO:0016874 ligase activity
GO:0019899 enzyme binding
GO:0046872 metal ion binding
Biological Process
GO:0008152 metabolic process
Cellular Component
GO:0005739 mitochondrion
Orthologs
Species
Homo sapiens
Mus musculus
Gene ID
Gene Order
Pathway Predictions based on Human Orthology Data
NETPATH
REACTOME
Propionyl-CoA catabolism pathway
Fatty acid, triacylglycerol, and ketone body metabolism pathway
Biotin transport and metabolism pathway
Metabolism of lipids and lipoproteins pathway
Defective HLCS causes multiple carboxylase deficiency pathway
Defective MUT causes methylmalonic aciduria mut type pathway
Defective MMAA causes methylmalonic aciduria type cblA pathway
Defective TCN2 causes hereditary megaloblastic anemia pathway
Mitochondrial Fatty Acid Beta-Oxidation pathway
Metabolism of water-soluble vitamins and cofactors pathway
Defective AMN causes hereditary megaloblastic anemia 1 pathway
Defective MTR causes methylmalonic aciduria and homocystinuria type cblG pathway
Defective LMBRD1 causes methylmalonic aciduria and homocystinuria type cblF pathway
Defective CD320 causes methylmalonic aciduria pathway
Defects in cobalamin (B12) metabolism pathway
Defective MMACHC causes methylmalonic aciduria and homocystinuria type cblC pathway
Defects in biotin (Btn) metabolism pathway
Defective BTD causes biotidinase deficiency pathway
Defective GIF causes intrinsic factor deficiency pathway
Defects in vitamin and cofactor metabolism pathway
Defective CUBN causes hereditary megaloblastic anemia 1 pathway
Defective MMADHC causes methylmalonic aciduria and homocystinuria type cblD pathway
Defective MTRR causes methylmalonic aciduria and homocystinuria type cblE pathway
Metabolism pathway
Defective MMAB causes methylmalonic aciduria type cblB pathway
Disease pathway
Metabolism of vitamins and cofactors pathway
Defective LMBRD1 causes methylmalonic aciduria and homocystinuria type cblF pathway
Disease pathway
Fatty acid, triacylglycerol, and ketone body metabolism pathway
Mitochondrial Fatty Acid Beta-Oxidation pathway
Metabolism pathway
Metabolism of lipids and lipoproteins pathway
Defective AMN causes hereditary megaloblastic anemia 1 pathway
Defective GIF causes intrinsic factor deficiency pathway
Defective MMAA causes methylmalonic aciduria type cblA pathway
Defective MMACHC causes methylmalonic aciduria and homocystinuria type cblC pathway
Defective MMADHC causes methylmalonic aciduria and homocystinuria type cblD pathway
Defective CD320 causes methylmalonic aciduria pathway
Defective MUT causes methylmalonic aciduria mut type pathway
Defects in biotin (Btn) metabolism pathway
Defective BTD causes biotidinase deficiency pathway
Defective MMAB causes methylmalonic aciduria type cblB pathway
Defective MTR causes methylmalonic aciduria and homocystinuria type cblG pathway
Defective MTRR causes methylmalonic aciduria and homocystinuria type cblE pathway
Defective CUBN causes hereditary megaloblastic anemia 1 pathway
Metabolism of vitamins and cofactors pathway
Metabolism of water-soluble vitamins and cofactors pathway
Biotin transport and metabolism pathway
Defects in vitamin and cofactor metabolism pathway
Defects in cobalamin (B12) metabolism pathway
Defective TCN2 causes hereditary megaloblastic anemia pathway
Propionyl-CoA catabolism pathway
Defective HLCS causes multiple carboxylase deficiency pathway
KEGG
Valine, leucine and isoleucine degradation pathway
Propanoate metabolism pathway
Glyoxylate and dicarboxylate metabolism pathway
Valine, leucine and isoleucine degradation pathway
Propanoate metabolism pathway
Glyoxylate and dicarboxylate metabolism pathway
INOH
Propanoate metabolism pathway
PID NCI
Cross-References
SwissProt
TrEMBL F6QNC4
UniProt Splice Variant
Entrez Gene 614302
UniGene Bt.55189
RefSeq NM_001083509
HUGO
OMIM
CCDS
HPRD
IMGT
EMBL DAAA02034753 DAAA02034754 DAAA02034755 DAAA02034756 DAAA02034757 DAAA02034758
GenPept
RNA Seq Atlas 614302