Coq2 Gene Summary [Mouse]
Enables 4-hydroxybenzoate polyprenyltransferase activity. Involved in ubiquinone biosynthetic process. Located in mitochondrion. Is extrinsic component of mitochondrial inner membrane. Is expressed in several structures, including brain; cardiovascular system; genitourinary system; gut; and integumental system. Human ortholog(s) of this gene implicated in primary coenzyme Q10 deficiency 1. Orthologous to human COQ2 (coenzyme Q2, polyprenyltransferase). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Coq2 |
| Official Name | coenzyme Q2 4-hydroxybenzoate polyprenyltransferase [Source:MGI Symbol;Acc:MGI:1919133] |
| Ensembl ID | ENSMUSG00000029319 |
| Bio databases IDs | |
| Aliases | coenzyme Q2 4-hydroxybenzoate polyprenyltransferase |
| Synonyms | 2310002F18Rik, CL640, coenzyme Q2 4-hydroxybenzoate polyprenyltransferase, coenzyme Q2, polyprenyltransferase, COQ10D1, MSA1, PHB:PPT, RGD1306722 |
| 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 Coq2 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- 4-hydroxybenzoate nonaprenyltransferase
- prenyltransferase
- enzyme
- UbiA family of prenyltransferases (PTases)
- UbiA prenyltransferase family
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- multiple system atrophy
- hereditary disorder
- primary coenzyme Q10 deficiency type 1
- susceptibility to multiple system atrophy type 1
- mitochondrial disorder
- nephrosis
- neonatal encephalopathy
regulated by
- TP53
- PERM1
- GPR15LG
regulates
- ubiquinone
- glycerol
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
- mitochondrial inner membrane
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Coq2 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- glycerol metabolic process
- isoprenoid biosynthetic process
- ubiquinone biosynthetic process
Cellular Component
Where in the cell the gene product is active
- mitochondrial inner membrane
Molecular Function
What the gene product does at the molecular level
- prenyltransferase activity
- 4-hydroxybenzoate decaprenyltransferase activity
- 4-hydroxybenzoate octaprenyltransferase activity
- 4-hydroxybenzoate nonaprenyltransferase activity