Ndufs3 Gene Summary [Mouse]
Enables NADH dehydrogenase (ubiquinone) activity. Involved in mitochondrial electron transport, NADH to ubiquinone and mitochondrial respiratory chain complex I assembly. Located in mitochondrion. Part of respiratory chain complex I. Is active in mitochondrial inner membrane. Is expressed in several structures, including alimentary system; ear; genitourinary system; integumental system; and nervous system. Human ortholog(s) of this gene implicated in nuclear type mitochondrial complex I deficiency 8. Orthologous to human NDUFS3 (NADH:ubiquinone oxidoreductase core subunit S3). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Processed Transcript |
| Official Symbol | Ndufs3 |
| Official Name | NADH:ubiquinone oxidoreductase core subunit S3 [Source:MGI Symbol;Acc:MGI:1915599] |
| Ensembl ID | ENSMUSG00000005510 |
| Bio databases IDs | |
| Aliases | NADH:ubiquinone oxidoreductase core subunit S3 |
| Synonyms | 0610010M09Rik, 30 Kda, ci, CI-30, CI-30kD, MC1DN8, NADH Dehydrogenase 30kDa, NADH dehydrogenase (ubiquinone) Fe-S protein 3, NADH:ubiquinone oxidoreductase core subunit S3, NADH-UBIQUINONE REDUCTASE |
| 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 Ndufs3 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- PRK06074
- NADH2 dehydrogenase
- enzyme
- NADH (or F420H2) dehydrogenase, subunit C
- protein binding
- NADH2 dehydrogenase (ubiquinone)
- Respiratory-chain NADH dehydrogenase, 30 Kd subunit
Pathways
Biological processes and signaling networks where the Ndufs3 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
- chromophobe renal cell carcinoma
- chromophobe renal cancer
- Mitochondrial complex I deficiency
- Huntington disease
- hereditary disorder
- nuclear type 8 mitochondrial complex I deficiency
- insulin resistance
- venous thromboembolism
- adult acute myeloid leukemia
- Leigh syndrome
regulates
- reactive oxygen species
- Mitochondrial ETC 1
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
- myelin enriched fraction
- Mitochondria
- mitochondrial membrane
- mitochondrial matrix
- mitochondrial inner membrane
- nuclear bodies
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Ndufs3 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- reactive oxygen species metabolic process
- transmembrane transport
- negative regulation of cell growth
- mitochondrial electron transport, NADH to ubiquinone
- mitochondrial respiratory chain complex I assembly
- aerobic respiration
- negative regulation of intrinsic apoptotic signaling pathway
- mitochondrial ATP synthesis coupled proton transport
- substantia nigra development
Cellular Component
Where in the cell the gene product is active
- mitochondrial membrane
- nuclear body
- respiratory chain complex I
- mitochondrial matrix
- mitochondrion
- mitochondrial inner membrane
Molecular Function
What the gene product does at the molecular level
- NADH dehydrogenase activity
- protein binding
- electron carrier activity
- NADH dehydrogenase (ubiquinone) activity