Coq8a Gene Summary [Mouse]
Enables enzyme activator activity. Involved in ubiquinone biosynthetic process. Located in mitochondrion. Is extrinsic component of mitochondrial inner membrane. Is expressed in heart and heart right ventricle. Used to study primary coenzyme Q10 deficiency 4. Human ortholog(s) of this gene implicated in primary coenzyme Q10 deficiency 4. Orthologous to human COQ8A (coenzyme Q8A). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Coq8a |
| Official Name | coenzyme Q8A [Source:MGI Symbol;Acc:MGI:1914676] |
| Ensembl ID | ENSMUSG00000026489 |
| Bio databases IDs | |
| Aliases | coenzyme Q8A |
| Synonyms | 4632432J16Rik, ADCK3, ARCA2, CABC1, coenzyme Q8A, COQ10D4, COQ8, LOC100506460, mKIAA0451, SCAR9 |
| 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 Coq8a often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- kinase
- protein kinase
- Protein Kinases, catalytic domain
- ADP binding
- protein binding
- enzyme activator activity
- ABC1 atypical kinase-like domain
Pathways
Biological processes and signaling networks where the Coq8a 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
- environmentally induced seizure
- hereditary disorder
- spinocerebellar ataxia recessive 9
- hyperlipidemia
- mitochondrial disorder
- COQ8A-related disorder
- primary coenzyme Q10 deficiency type 1
- cataract
- cerebellar ataxia
- seizures
regulates
- ubiquinone
- ALOX15
role in cell
- expression in
- cell death
- abnormal morphology
- ferroptosis
- degeneration
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 Coq8a gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- phosphorylation
- protein phosphorylation
- ubiquinone biosynthetic process
Cellular Component
Where in the cell the gene product is active
- membrane
- mitochondrion
Molecular Function
What the gene product does at the molecular level
- ATP binding
- protein binding
- protein kinase activity
- ADP binding
- kinase activity