Version 5.4
Homo sapiens Protein: PCCB
Summary
InnateDB Protein IDBP-481630.4
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol PCCB
Protein Name propionyl CoA carboxylase, beta polypeptide
Synonyms
Species Homo sapiens
Ensembl Protein ENSP00000419027
InnateDB Gene IDBG-57834 (PCCB)
Protein Structure
UniProt Annotation
Function
Subcellular Localization Mitochondrion matrix {ECO:0000269PubMed:16023992}.
Disease Associations Propionic acidemia type II (PA-2) [MIM:606054]: Life- threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. {ECO:0000269PubMed:10447268, ECO:0000269PubMed:12189489, ECO:0000269PubMed:12559849, ECO:0000269PubMed:15059621, ECO:0000269PubMed:2154743, ECO:0000269PubMed:8411997, ECO:0000269PubMed:9683601}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity
Comments
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:0004658 propionyl-CoA carboxylase activity
GO:0005524 ATP binding
GO:0016874 ligase activity
Biological 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: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
Protein Structure and Domains
PDB ID
InterPro IPR000022 Carboxyl transferase
IPR011762 Acetyl-coenzyme A carboxyltransferase, N-terminal
IPR011763 Acetyl-coenzyme A carboxyltransferase, C-terminal
IPR029045 ClpP/crotonase-like domain
PFAM PF01039
PRINTS
PIRSF
SMART
TIGRFAMs
Post-translational Modifications
Modification
Cross-References
SwissProt P05166
PhosphoSite PhosphoSite-P05166
TrEMBL Q8WVH4
UniProt Splice Variant
Entrez Gene 5096
UniGene Hs.736024
RefSeq NP_001171485
HUGO HGNC:8654
OMIM 232050
CCDS CCDS54643
HPRD 01982
IMGT
EMBL AC069524 AJ006487 AJ006488 AJ006489 AJ006490 AJ006491 AJ006492 AJ006493 AJ006494 AJ006495 AJ006496 AJ006497 AJ006498 AJ006499 AK295312 BC013768 BC018013 BC053661 CH471052 M13573 M31167 M31169 S67325 X73424
GenPept AAA60036 AAA60037 AAA60038 AAB28900 AAH13768 AAH18013 AAH53661 BAH12030 CAA07066 CAA51825 EAW79118

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