Surf1 Gene Summary [Mouse]
Enables cytochrome-c oxidase activity. Predicted to be involved in mitochondrial cytochrome c oxidase assembly. Located in mitochondrion. Is expressed in brain; embryo; and skeletal muscle. Used to study Leigh disease. Human ortholog(s) of this gene implicated in Charcot-Marie-Tooth disease type 4K; Leigh disease; and mitochondrial complex IV deficiency nuclear type 1. Orthologous to human SURF1 (SURF1 cytochrome c oxidase assembly factor). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Retained Intron |
| Official Symbol | Surf1 |
| Official Name | surfeit gene 1 [Source:MGI Symbol;Acc:MGI:98443] |
| Ensembl ID | ENSMUSG00000015790 |
| Bio databases IDs | |
| Aliases | surfeit gene 1 |
| Synonyms | 0610010F23Rik, Ab1-205, CMT4K, LOC100912008, MC4DN1, SHY1, Surf, SURF1 cytochrome c oxidase assembly factor, SURF1, cytochrome c oxidase assembly factor, surfeit gene 1 |
| 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 Surf1 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- cytochrome-c oxidase
- SURF1
- enzyme
- protein binding
Pathways
Biological processes and signaling networks where the Surf1 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
- nuclear type 1 mitochondrial complex iv deficiency
- Leigh syndrome
- hereditary disorder
- mitochondrial disorder
- fatal infantile cardioencephalomyopathy due to cytochrome C oxidase deficiency 2
- Charcot-Marie-Tooth disease type 4K
- cytochrome c oxidase deficiency
- demyelinating peripheral neuropathy
- autosomal recessive nonsyndromic mental retardation
role in cell
- growth
- function
- size
- respiration
- excitotoxicity
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 Surf1 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- mitochondrial respiratory chain complex IV assembly
- aerobic respiration
- respiratory chain complex IV assembly
Cellular Component
Where in the cell the gene product is active
- mitochondrial respiratory chain
- mitochondrion
Molecular Function
What the gene product does at the molecular level
- protein binding
- cytochrome-c oxidase activity