Lonp1 Gene Summary [Mouse]
Enables several functions, including ATP hydrolysis activity; ATP-dependent peptidase activity; and DNA binding activity. Acts upstream of or within negative regulation of insulin receptor signaling pathway; proteolysis; and regulation of peptidyl-tyrosine phosphorylation. Located in mitochondrion. Is expressed in several structures, including adrenal medulla; genitourinary system; gut; lung; and submandibular gland primordium. Human ortholog(s) of this gene implicated in CODAS syndrome. Orthologous to human LONP1 (lon peptidase 1, mitochondrial). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Nonsense Mediated Decay |
| Official Symbol | Lonp1 |
| Official Name | lon peptidase 1, mitochondrial [Source:MGI Symbol;Acc:MGI:1921392] |
| Ensembl ID | ENSMUSG00000041168 |
| Bio databases IDs | |
| Aliases | lon peptidase 1, mitochondrial |
| Synonyms | 1200017E13Rik, CODASS, hLON, LON, LonHS, LONM, LONP, lon peptidase 1, mitochondrial, Mitochondrial LON Protease, PIM1, Protease, serine, 15, PRSS15 |
| 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 Lonp1 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- pleckstrin Homology binding region
- ATP-dependent protease La (LON) substrate-binding domain
- ADP binding
- peptidase
- ATP binding
- Lon protease (S16) C-terminal proteolytic domain
- enzyme binding
- protein binding
- ATP-dependent peptidase
- sequence-specific DNA binding
- identical protein binding
- ATPase
- single-stranded RNA binding
- PH-domain binding
- DNA binding
- Found in ATP-dependent protease La (LON)
- AAA domain (dynein-related subfamily)
- endopeptidase La
- ATPase family associated with various cellular activities (AAA)
- single-stranded DNA binding
- P-loop containing Nucleoside Triphosphate Hydrolases
- ATPases associated with a variety of cellular activities
Pathways
Biological processes and signaling networks where the Lonp1 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
- neoplasia
- chronic lymphocytic leukemia
- mantle cell lymphoma
- breast cancer
- bladder cancer
- CODAS syndrome
- Leigh syndrome
- lung squamous cell carcinoma
- squamous cell lung cancer
- Alzheimer disease
regulated by
- hemin
- brefeldin A
- OMA1
- bardoxolone methyl
- GADD45GIP1
- metformin
- CHCHD3
- Pick1
- Yersinia pestis strain KIM D27
- RAW 264.7 cells
role in cell
- expression in
- cell death
- accumulation in
- differentiation
- proliferation
- accumulation
- upregulation in
- mitochondrial respiration in
- quantity
- growth
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
- Nucleus
- Mitochondria
- cytosol
- mitochondrial matrix
- mitochondrial nucleoids
- nucleoplasm
- peroxisomes
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Lonp1 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- mitochondrial protein catabolic process
- oxidation-dependent protein catabolic process
- protein catabolic process
- mitochondrial DNA metabolic process
- negative regulation of insulin receptor signaling pathway
- mitochondrion organization
- mitochondrial genome maintenance
- regulation of peptidyl-tyrosine phosphorylation
- misfolded or incompletely synthesized protein catabolic process
- response to hypoxia
- chaperone-mediated protein complex assembly
- cellular response to oxidative stress
- proteolysis involved in cellular protein catabolic process
Cellular Component
Where in the cell the gene product is active
- mitochondrial matrix
- mitochondrion
- membrane
- cytosol
- mitochondrial nucleoid
- nucleoplasm
Molecular Function
What the gene product does at the molecular level
- ATPase activity
- identical protein binding
- serine-type endopeptidase activity
- sequence-specific DNA binding
- ADP binding
- ATP-dependent peptidase activity
- mitochondrial RNA polymerase regulatory region DNA binding
- PH domain binding
- single-stranded DNA binding
- ATP binding
- DNA polymerase binding
- protein binding
- G-quadruplex DNA binding
- single-stranded RNA binding
- insulin receptor substrate binding