propionyl CoA carboxylase, beta polypeptide
Symbol
PCCB
(may also be known as: None)
Organism
Human
Description
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]
Orthologs
| Symbol | Species | |
|---|---|---|
| pccb-1 | Caenorhabditis elegans | |
| pccb | Danio rerio | |
| Pccb | Mus musculus | |
| Pccb | Rattus norvegicus |
Links to external resources
Changes associated with this gene
GO Terms
| GO ID | GO Term | GO Category | |
|---|---|---|---|
| GO:0006633 | fatty acid biosynthetic process | biological_process | |
| GO:0006635 | fatty acid beta-oxidation | biological_process | |
| GO:0019626 | short-chain fatty acid catabolic process | biological_process | |
| GO:0044255 | cellular lipid metabolic process | biological_process | |
| GO:0044281 | small molecule metabolic process | biological_process | |
| GO:0005739 | mitochondrion | cellular_component | |
| GO:0005759 | mitochondrial matrix | cellular_component | |
| GO:0009317 | acetyl-CoA carboxylase complex | cellular_component | |
| GO:0000166 | nucleotide binding | molecular_function | |
| GO:0003989 | acetyl-CoA carboxylase activity | molecular_function | |
| GO:0004658 | propionyl-CoA carboxylase activity | molecular_function | |
| GO:0005524 | ATP binding | molecular_function | |
| GO:0016874 | ligase activity | molecular_function | |