Pcca Gene Summary [Mouse]
Enables propionyl-CoA carboxylase activity. Predicted to be involved in lipid catabolic process. Located in mitochondrion. Is expressed in several structures, including alimentary system; nervous system; orbito-sphenoid; sensory organ; and urinary system. Used to study propionic acidemia. Human ortholog(s) of this gene implicated in amino acid metabolic disorder and propionic acidemia. Orthologous to human PCCA (propionyl-CoA carboxylase subunit alpha). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Retained Intron |
| Official Symbol | Pcca |
| Official Name | propionyl-Coenzyme A carboxylase, alpha polypeptide [Source:MGI Symbol;Acc:MGI:97499] |
| Ensembl ID | ENSMUSG00000041650 |
| Bio databases IDs | |
| Aliases | propionyl-Coenzyme A carboxylase, alpha polypeptide |
| Synonyms | alphaPCC, Propionyl coa carboxylase alpha, propionyl-CoA carboxylase subunit alpha, propionyl-CoA carboxylase subunit α, Propionyl coa carboxylase α, propionyl-Coenzyme A carboxylase, alpha polypeptide, propionyl-Coenzyme A carboxylase, α polypeptide, αPCC |
| Species | Mouse, Mus musculus |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in mouse Pcca often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- Propionyl-coenzyme A carboxylase BT domain
- biotin binding
- enzyme binding
- Biotin carboxylase C-terminal domain
- enzyme
- protein binding
- D-ala D-ala ligase C-terminus
- Biotinyl_lipoyl_domains
- acetyl-CoA carboxylase, biotin carboxylase subunit
- Carbamoyl-phosphate synthase L chain, ATP binding domain
- Biotin carboxylase, N-terminal domain
- Biotin-requiring enzyme
- propionyl-CoA carboxylase
- PRK12999
Pathways
Biological processes and signaling networks where the Pcca gene in mouse plays a role, providing insight into its function and relevance in health or disease.
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- non-insulin-dependent diabetes mellitus
- propionic acidemia
- early onset hypertension
- dry skin
- refractive error
- ketoaciduria
- uterine leiomyoma
- leiomyomatosis
- myopia
- hepatic steatosis
regulates
- ammonia
- amino acids
- fatty acid
- glycogen
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Cytoplasm
- Mitochondria
- cytosol
- mitochondrial matrix
- plasma
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Pcca gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- short-chain fatty acid catabolic process
- branched chain family amino acid metabolic process
- fatty acid metabolic process
Cellular Component
Where in the cell the gene product is active
- mitochondrial matrix
- mitochondrion
- cytosol
Molecular Function
What the gene product does at the molecular level
- ATP binding
- propionyl-CoA carboxylase activity
- protein binding
- metal ion binding
- biotin binding
- enzyme binding