Tmem126b Gene Summary [Mouse]
Acts upstream of or within mitochondrion organization and response to food. Predicted to be located in mitochondrial membrane. Predicted to be active in mitochondrion. Human ortholog(s) of this gene implicated in nuclear type mitochondrial complex I deficiency 29. Orthologous to human TMEM126B (transmembrane protein 126B). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Tmem126b |
| Official Name | transmembrane protein 126B [Source:MGI Symbol;Acc:MGI:1915722] |
| Ensembl ID | ENSMUSG00000030614 |
| Bio databases IDs | |
| Aliases | transmembrane protein 126B |
| Synonyms | 1110001A23Rik, HT007, MC1DN29, RGD1308371, transmembrane protein 126B |
| 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 Tmem126b often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- Transmembrane protein 126
- protein binding
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 29 mitochondrial complex I deficiency
- ischemic stroke
- Mitochondrial complex I deficiency
- mitochondrial disorder
- nuclear type 1 mitochondrial complex I deficiency
regulates
- Mitochondrial ETC 1
role in cell
- organization
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 Tmem126b gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- response to food
- mitochondrial respiratory chain complex I assembly
Cellular Component
Where in the cell the gene product is active
- mitochondrion
- mitochondrial inner membrane
Molecular Function
What the gene product does at the molecular level
- protein binding