Version 5.4
Mus musculus Gene: Pcca
Summary
InnateDB Gene IDBG-188896.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol Pcca
Gene Name propionyl-Coenzyme A carboxylase, alpha polypeptide
Synonyms C79630
Species Mus musculus
Ensembl Gene ENSMUSG00000041650
Encoded Proteins
IDBP-188898
propionyl-Coenzyme A carboxylase, alpha polypeptide
IDBP-542119
propionyl-Coenzyme A carboxylase, alpha polypeptide
IDBP-536346
propionyl-Coenzyme A carboxylase, alpha polypeptide
IDBP-716246
IDBP-716147
IDBP-718111
IDBP-717466
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 14:122534324-122891100
Strand Forward strand
Band E5
Transcripts
ENSMUST00000038374 ENSMUSP00000038763
ENSMUST00000148172
ENSMUST00000154206 ENSMUSP00000120027
ENSMUST00000135578 ENSMUSP00000123422
ENSMUST00000143240
ENSMUST00000147018 ENSMUSP00000135557
ENSMUST00000152383 ENSMUSP00000135266
ENSMUST00000156755
ENSMUST00000128893
ENSMUST00000132444 ENSMUSP00000135087
ENSMUST00000140517
ENSMUST00000138836
ENSMUST00000177312 ENSMUSP00000135665
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 6 experimentally validated interaction(s) in this database.
They are also associated with 3 interaction(s) predicted by orthology.
Experimentally validated
Total 6 [view]
Protein-Protein 6 [view]
Protein-DNA 0
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Predicted by orthology
Total 3 [view]
Gene Ontology

Molecular Function
Accession GO Term
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
GO:0009405 pathogenesis
GO:0019836 hemolysis by symbiont of host erythrocytes
Cellular Component
GO:0005576 extracellular region
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
Orthologs
Species
Homo sapiens
Bos taurus
Gene ID
Gene Order
ENSG00000175198
View Gene Order
ENSBTAG00000010672
Not yet available
Pathways
NETPATH
REACTOME
REACT_189088
Defective LMBRD1 causes methylmalonic aciduria and homocystinuria type cblF pathway
REACT_189085
Disease pathway
REACT_256522
Fatty acid, triacylglycerol, and ketone body metabolism pathway
REACT_233858
Mitochondrial Fatty Acid Beta-Oxidation pathway
REACT_188937
Metabolism pathway
REACT_232291
Metabolism of lipids and lipoproteins pathway
REACT_189089
Defective AMN causes hereditary megaloblastic anemia 1 pathway
REACT_189086
Defective GIF causes intrinsic factor deficiency pathway
REACT_189075
Defective MMAA causes methylmalonic aciduria type cblA pathway
REACT_189076
Defective MMACHC causes methylmalonic aciduria and homocystinuria type cblC pathway
REACT_189078
Defective MMADHC causes methylmalonic aciduria and homocystinuria type cblD pathway
REACT_189098
Defective CD320 causes methylmalonic aciduria pathway
REACT_189097
Defective MUT causes methylmalonic aciduria mut type pathway
REACT_189096
Defects in biotin (Btn) metabolism pathway
REACT_189095
Defective BTD causes biotidinase deficiency pathway
REACT_189093
Defective MMAB causes methylmalonic aciduria type cblB pathway
REACT_189092
Defective MTR causes methylmalonic aciduria and homocystinuria type cblG pathway
REACT_189091
Defective MTRR causes methylmalonic aciduria and homocystinuria type cblE pathway
REACT_189090
Defective CUBN causes hereditary megaloblastic anemia 1 pathway
REACT_254651
Metabolism of vitamins and cofactors pathway
REACT_238663
Metabolism of water-soluble vitamins and cofactors pathway
REACT_206176
Biotin transport and metabolism pathway
REACT_189110
Defects in vitamin and cofactor metabolism pathway
REACT_189116
Defects in cobalamin (B12) metabolism pathway
REACT_189114
Defective TCN2 causes hereditary megaloblastic anemia pathway
REACT_253750
Propionyl-CoA catabolism pathway
REACT_189100
Defective HLCS causes multiple carboxylase deficiency pathway
KEGG
mmu00280
Valine, leucine and isoleucine degradation pathway
mmu00640
Propanoate metabolism pathway
mmu00630
Glyoxylate and dicarboxylate metabolism pathway
INOH
PID NCI
Pathway Predictions based on Human Orthology Data
NETPATH
REACTOME
REACT_993
Propionyl-CoA catabolism pathway
REACT_22279
Fatty acid, triacylglycerol, and ketone body metabolism pathway
REACT_11153
Biotin transport and metabolism pathway
REACT_22258
Metabolism of lipids and lipoproteins 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_169120
Defective TCN2 causes hereditary megaloblastic anemia pathway
REACT_1473
Mitochondrial Fatty Acid Beta-Oxidation pathway
REACT_11238
Metabolism of water-soluble vitamins and cofactors 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_11193
Metabolism of vitamins and cofactors pathway
KEGG
hsa00280
Valine, leucine and isoleucine degradation pathway
hsa00640
Propanoate metabolism pathway
hsa00630
Glyoxylate and dicarboxylate metabolism pathway
INOH
INOH website
Propanoate metabolism pathway
PID NCI
Cross-References
SwissProt Q91ZA3
TrEMBL Q3UGC8
UniProt Splice Variant
Entrez Gene 110821
UniGene Mm.23876 Mm.482957
RefSeq NM_144844 XM_006518432
OMIM
CCDS CCDS27350
HPRD
IMGT
MGI ID MGI:97499
MGI Symbol Pcca
EMBL AK148002 AY046947 BC006915 BC049802 CH466544
GenPept AAH06915 AAH49802 AAL02364 BAE28281 EDL00631
RNA Seq Atlas 110821

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