| Gene Symbol | LONP1 |
| Full Name | Lon Peptidase 1 |
| Chromosome | 19p13.3 |
| Protein Type | Protease |
| Function | LONP1 (Lon Peptidase 1, Mitochondrial) is a nuclear-encoded mitochondrial ATP-dependent protease essential for mitochondrial protein quality control. |
| Primary Expression | most tissues, with high expression in metabolically active tissues including brain, heart, and muscle |
| Subcellular Localization | the mitochondrial matrix. Its expression can be upregulated under conditions of mitochondrial stress |
| Pathways | Mitochondrial Proteostasis |
| UniProt ID | [P36776](https://www.uniprot.org/uniprot/P36776) |
| Ensembl ID | ENSG00000125726 |
| OMIM | 607440 |
| GeneCards | LONP1 |
| Human Protein Atlas | LONP1 |
| Associated Diseases | Aging, Als, frontotemporal dementia, Ftd, Lymphoma, Neurodegeneration |
| Interactions | AGING, ALS, AMYOTROPHIC LATERAL SCLEROSIS, APOPTOSIS, ATF3, ATP |
| KG Connections | 308 knowledge graph edges |
| Databases | GeneCardsNCBI GeneHPASTRING |
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