Lars2 Gene Summary [Mouse]
Predicted to enable leucine-tRNA ligase activity. Predicted to be involved in leucyl-tRNA aminoacylation and mitochondrial translation. Located in mitochondrion. Is expressed in lens and pancreas epithelium. Human ortholog(s) of this gene implicated in Perrault syndrome. Orthologous to human LARS2 (leucyl-tRNA synthetase 2, mitochondrial). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Lars2 |
| Official Name | leucyl-tRNA synthetase, mitochondrial [Source:MGI Symbol;Acc:MGI:2142973] |
| Ensembl ID | ENSMUSG00000035202 |
| Bio databases IDs | |
| Aliases | leucyl-tRNA synthetase, mitochondrial |
| Synonyms | HLASA, Kiaa0028, Leucine--tRNA ligase, leucyl-tRNA synthetase 2, mitochondrial, leucyl-tRNA synthetase, mitochondrial, LEURS, MITOCHONDRIAL TRNA-LEU, mtLeuRS, PRLTS4 |
| 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 Lars2 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- Leucyl-tRNA synthetase, Domain 2
- isoleucyl-tRNA synthetase
- leucyl-tRNA synthetase, eubacterial and mitochondrial family
- enzyme
- leucine-tRNA ligase
- nucleotidyl transferase superfamily
- valyl-tRNA synthetase
- Anticodon-binding domain of tRNA ligase
- class Ia aminoacyl tRNA synthetases anticodon binding domain
- tRNA synthetases class I (I, L, M and V)
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- COVID-19
- hydrops, lactic acidosis, and sideroblastic anemia
- Perrault syndrome type 4
- maturity-onset diabetes of the young
- idiopathic scoliosis
- gonadal dysgenesis with auditory dysfunction, autosomal recessive inheritance
- familial nonsyndromic hearing impairment
- myelodysplastic syndrome
- mitochondrial myopathy
regulates
- tRNA-Leu
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
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Lars2 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- tRNA aminoacylation for protein translation
- leucyl-tRNA aminoacylation
- mitochondrial translation
Cellular Component
Where in the cell the gene product is active
- mitochondrial matrix
- mitochondrion
Molecular Function
What the gene product does at the molecular level
- ATP binding
- leucine-tRNA ligase activity
- aminoacyl-tRNA editing activity