Sco2 Gene Summary [Mouse]
Predicted to enable protein-disulfide reductase activity. Acts upstream of or within several processes, including copper ion homeostasis; respiratory chain complex IV assembly; and response to activity. Located in mitochondrion. Is expressed in several structures, including alimentary system; aorta; blastocyst; gonad; and hemolymphoid system gland. Used to study cytochrome-c oxidase deficiency disease. Human ortholog(s) of this gene implicated in cytochrome-c oxidase deficiency disease; hypertrophic cardiomyopathy; mitochondrial complex IV deficiency nuclear type 2; and myopia. Orthologous to human SCO2 (synthesis of cytochrome C oxidase 2). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Sco2 |
| Official Name | SCO2 cytochrome c oxidase assembly protein [Source:MGI Symbol;Acc:MGI:3818630] |
| Ensembl ID | ENSMUSG00000091780 |
| Bio databases IDs | |
| Aliases | SCO2 cytochrome c oxidase assembly protein |
| Synonyms | CEMCOX1, ECGF1, Gliostatin, MC4DN2, MYP6, PD-ECGF, SCO1L, SCO2 cytochrome c oxidase assembly protein, synthesis of cytochrome C oxidase 2, TdRPase, TP, TYMP |
| 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 Sco2 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- protein disulfide oxidoreductase
- Thioredoxin-like
- Protein Disulfide Oxidoreductases and Other Proteins with a Thioredoxin fold
- SCO1/SenC
- AhpC/TSA family
- enzyme
- protein binding
Pathways
Biological processes and signaling networks where the Sco2 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
- fatal infantile cardioencephalomyopathy due to cytochrome C oxidase deficiency 1
- psoriasis
- systemic lupus erythematosus
- myopia type 6
- lung adenocarcinoma
- lung adenocarcinoma formation
- Leigh syndrome
- mitochondrial DNA depletion syndrome 1 (MNGIE type)
- pemphigus foliaceus
- primary dilated cardiomyopathy
role in cell
- necrosis
- respiration
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
- myofibrils
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Sco2 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
- eye development
- cellular copper ion homeostasis
- in utero embryonic development
- respiratory electron transport chain
- response to activity
- muscle system process
Cellular Component
Where in the cell the gene product is active
- mitochondrial matrix
- mitochondrion
- mitochondrial inner membrane
- myofibril
Molecular Function
What the gene product does at the molecular level
- protein disulfide oxidoreductase activity
- protein binding
- copper ion binding
- copper chaperone activity