Version 5.4
Homo sapiens Gene: PCCB
Summary
InnateDB Gene IDBG-57834.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol PCCB
Gene Name propionyl CoA carboxylase, beta polypeptide
Synonyms
Species Homo sapiens
Ensembl Gene ENSG00000114054
Encoded Proteins
IDBP-57836
propionyl CoA carboxylase, beta polypeptide
IDBP-477005
propionyl CoA carboxylase, beta polypeptide
IDBP-479186
propionyl CoA carboxylase, beta polypeptide
IDBP-480487
propionyl CoA carboxylase, beta polypeptide
IDBP-480169
propionyl CoA carboxylase, beta polypeptide
IDBP-481341
propionyl CoA carboxylase, beta polypeptide
IDBP-473485
propionyl CoA carboxylase, beta polypeptide
IDBP-482498
propionyl CoA carboxylase, beta polypeptide
IDBP-483966
propionyl CoA carboxylase, beta polypeptide
IDBP-481630
propionyl CoA carboxylase, beta polypeptide
IDBP-478049
propionyl CoA carboxylase, beta polypeptide
IDBP-484425
propionyl CoA carboxylase, beta polypeptide
IDBP-483947
propionyl CoA carboxylase, beta polypeptide
IDBP-483476
propionyl CoA carboxylase, beta polypeptide
IDBP-480182
propionyl CoA carboxylase, beta polypeptide
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary The protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). 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 3:136250306-136337896
Strand Forward strand
Band q22.3
Transcripts
ENST00000251654 ENSP00000251654
ENST00000490504 ENSP00000418307
ENST00000483687 ENSP00000420639
ENST00000468777 ENSP00000419129
ENST00000462637 ENSP00000420391
ENST00000466072 ENSP00000420158
ENST00000484181 ENSP00000417937
ENST00000482086 ENSP00000417253
ENST00000471595 ENSP00000417549
ENST00000469217 ENSP00000419027
ENST00000465423 ENSP00000419263
ENST00000478469 ENSP00000420759
ENST00000474833
ENST00000462542 ENSP00000419563
ENST00000494742 ENSP00000418020
ENST00000459873 ENSP00000419293
ENST00000475214
ENST00000465176
ENST00000473073
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 11 experimentally validated interaction(s) in this database.
Experimentally validated
Total 11 [view]
Protein-Protein 11 [view]
Protein-DNA 0
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Gene Ontology

Molecular Function
Accession GO Term
GO:0003989 acetyl-CoA carboxylase activity
GO:0004658 propionyl-CoA carboxylase activity
GO:0005524 ATP binding
GO:0016874 ligase activity
Biological Process
GO:0006633 fatty acid biosynthetic process
GO:0006635 fatty acid beta-oxidation
GO:0006766 vitamin metabolic process
GO:0006767 water-soluble vitamin metabolic process
GO:0006768 biotin metabolic process
GO:0008152 metabolic process
GO:0019626 short-chain fatty acid catabolic process
GO:0044255 cellular lipid metabolic process
GO:0044281 small molecule metabolic process
Cellular Component
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
GO:0005829 cytosol
GO:0009317 acetyl-CoA carboxylase complex
Orthologs
Species
Mus musculus
Bos taurus
Gene ID
Gene Order
ENSMUSG00000032527
View Gene Order
ENSBTAG00000015221
Not yet available
Pathways
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
TrEMBL
UniProt Splice Variant
Entrez Gene
UniGene Hs.63788 Hs.736024
RefSeq NM_000532 NM_001178014
HUGO
OMIM
CCDS CCDS3089 CCDS54643
HPRD 01982
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