Ndufs2 Gene Summary [Mouse]
Enables NADH dehydrogenase (ubiquinone) activity and oxygen sensor activity. Involved in several processes, including cellular response to oxygen levels; gliogenesis; 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; integumental system; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in Leigh disease; hypertrophic cardiomyopathy; inherited metabolic disorder; multiple sclerosis; and nuclear type mitochondrial complex I deficiency 6. Orthologous to human NDUFS2 (NADH:ubiquinone oxidoreductase core subunit S2). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Retained Intron |
| Official Symbol | Ndufs2 |
| Official Name | NADH:ubiquinone oxidoreductase core subunit S2 [Source:MGI Symbol;Acc:MGI:2385112] |
| Ensembl ID | ENSMUSG00000013593 |
| Bio databases IDs | |
| Aliases | NADH:ubiquinone oxidoreductase core subunit S2 |
| Synonyms | CI-49, CI-49kD, Complex I-49KD, LHONAR2, LOC103690046, MC1DN6, Nadh-coenzyme q reductase, Nadh Ubiquinone Oxidoreductase 49 Kda Subunit, NADH:ubiquinone oxidoreductase core subunit S2 |
| 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 Ndufs2 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- ubiquitin protein ligase binding
- oxygen sensor
- NADH2 dehydrogenase
- NADH dehydrogenase I, D subunit
- enzyme
- protein binding
- NADH2 dehydrogenase (ubiquinone)
- Respiratory-chain NADH dehydrogenase, 49 Kd subunit
- NADH-quinone oxidoreductase subunit D
Pathways
Biological processes and signaling networks where the Ndufs2 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
- autosomal recessive Leber-like hereditary optic neuropathy type 2
- Mitochondrial complex I deficiency
- insulin resistance
- Huntington disease
- pancreatic cancer
- nuclear type 6 mitochondrial complex I deficiency
- Leigh syndrome
- chromophobe renal cell carcinoma
- chromophobe renal cancer
- pancreatic adenocarcinoma
regulates
- oxygen
- Mitochondrial ETC 1
role in cell
- proliferation
- function
- ferroptosis
- consumption in
- autophagy in
- autophagy
- radiosensitivity
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
- nucleoplasm
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Ndufs2 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- response to oxidative stress
- transmembrane transport
- cellular response to oxygen levels
- gliogenesis
- mitochondrial electron transport, NADH to ubiquinone
- neural precursor cell proliferation
- neurogenesis
- mitochondrial respiratory chain complex I assembly
- aerobic respiration
- mitochondrial ATP synthesis coupled electron transport
- mitochondrial ATP synthesis coupled proton transport
Cellular Component
Where in the cell the gene product is active
- respiratory chain complex I
- mitochondrial matrix
- mitochondrion
- mitochondrial inner membrane
- nucleoplasm
Molecular Function
What the gene product does at the molecular level
- protein binding
- oxygen sensor activity
- NADH dehydrogenase activity
- ubiquitin protein ligase binding
- quinone binding
- metal ion binding
- NAD binding
- electron carrier activity
- NADH dehydrogenase (ubiquinone) activity
- 4 iron, 4 sulfur cluster binding