Coq4 Gene Summary [Mouse]
Predicted to enable carboxy-lyase activity. Predicted to be involved in ubiquinone biosynthetic process. Located in mitochondrion. Is expressed in several structures, including brain; colon; gonad; heart; and lung. Human ortholog(s) of this gene implicated in primary coenzyme Q10 deficiency 7 and spastic ataxia. Orthologous to human COQ4 (coenzyme Q4). [provided by Alliance of Genome Resources, Apr 2025]
Details
| Type | Nonsense Mediated Decay |
| Official Symbol | Coq4 |
| Official Name | coenzyme Q4 [Source:MGI Symbol;Acc:MGI:1098826] |
| Ensembl ID | ENSMUSG00000026798 |
| Bio databases IDs | |
| Aliases | coenzyme Q4 |
| Synonyms | CGI-92, coenzyme Q4, COQ10D7, D2ERTD97E, SPAX10, Ubiqinone biosynthase |
| 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 Coq4 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- carboxy-lyase
- enzyme
- protein binding
- Coenzyme Q (ubiquinone) biosynthesis protein Coq4
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- autosomal recessive spastic ataxia type 10
- hereditary spastic ataxia
- primary coenzyme Q10 deficiency type 7
- developmental disorder
- mitochondrial disorder
- COQ4-related disorder
- hereditary disorder
- breast cancer
regulated by
- TP53
- PARL
regulates
- ubiquinone
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 matrix
- mitochondrial inner membrane
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Coq4 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- ubiquinone biosynthetic process
Cellular Component
Where in the cell the gene product is active
- mitochondrion
- macromolecular complex
- extrinsic to mitochondrial inner membrane
- mitochondrial inner membrane
Molecular Function
What the gene product does at the molecular level
- protein binding