Entity Detail — Knowledge Graph Node
This page aggregates everything SciDEX knows about LONP1: its mechanistic relationships (Knowledge Graph edges), hypotheses targeting it, analyses mentioning it, and supporting scientific papers. The interactive graph below shows its immediate neighbors. All content is AI-synthesized from peer-reviewed literature.
LONP1 is a gene implicated in neurodegeneration research. Key relationships include: associated with, therapeutic target, activates. Associated with ALS, Aging, Als. Connected to 213 entities in the SciDEX knowledge graph.
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| 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, AND, APOPTOSIS, ATF3 |
| KG Connections | 311 knowledge graph edges |
| Databases | GeneCardsNCBI GeneHPASTRING |
Knowledge base pages for this entity
graph TD
LONP1["LONP1"]
LONP1 -->|"target for"| Neurodegeneration["Neurodegeneration"]
LONP1 -->|"target for"| Aging["Aging"]
LONP1 -->|"target for"| Ms["Ms"]
LONP1 -->|"expressed in"| Carcinoma["Carcinoma"]
LONP1 -->|"expressed in"| Tumor["Tumor"]
LONP1 -->|"inhibits"| Heart_Failure["Heart Failure"]
LONP1 -->|"transports"| ALS["ALS"]
LONP1 -->|"target for"| BDNF["BDNF"]
ELAVL2["ELAVL2"] -->|"target for"| LONP1
C1Q["C1Q"] -->|"target for"| LONP1
MTOR["MTOR"] -->|"target for"| LONP1
RAB11["RAB11"] -->|"target for"| LONP1
STX17["STX17"] -->|"target for"| LONP1
RAB5["RAB5"] -->|"target for"| LONP1
SNAP29["SNAP29"] -->|"target for"| LONP1
IGF2BP1["IGF2BP1"] -->|"target for"| LONP1
PI3K["PI3K"] -->|"target for"| LONP1
BDNF -->|"target for"| LONP1| Target | Relation | Type | Str |
|---|---|---|---|
| Mitochondrial Proteostasis | involved_in | process | 0.85 |
| Proteostasis | involved_in | process | 0.85 |
| mitochondrial proteostasis | regulates | process | 0.85 |
| AKR1B10 | downstream_of | gene | 0.80 |
| Als | activates | disease | 0.75 |
| Aging | associated_with | disease | 0.65 |
| Ftd | associated_with | disease | 0.65 |
| Neurodegeneration | associated_with | disease | 0.65 |
| Aging | activates | disease | 0.65 |
| Inflammation | activates | disease | 0.65 |
| Neurodegeneration | therapeutic_target | disease | 0.65 |
| Aging | therapeutic_target | disease | 0.65 |
| Ms | therapeutic_target | disease | 0.65 |
| Als | associated_with | disease | 0.65 |
| Carcinoma | expressed_in | disease | 0.65 |
| Metabolic Syndrome | activates | disease | 0.65 |
| ALS | transports | disease | 0.65 |
| Parkinson | interacts_with | disease | 0.65 |
| Neurodegeneration | interacts_with | disease | 0.65 |
| Fibrosis | activates | disease | 0.65 |
| Ms | expressed_in | disease | 0.65 |
| Aging | encodes | disease | 0.65 |
| Heart Failure | inhibits | disease | 0.65 |
| Ms | inhibits | disease | 0.65 |
| Hepatitis | activates | disease | 0.65 |
| Als | interacts_with | disease | 0.65 |
| Als | transports | disease | 0.65 |
| Neurodegeneration | encodes | disease | 0.65 |
| Tumor | expressed_in | disease | 0.65 |
| Amyotrophic Lateral Sclerosis | activates | disease | 0.65 |
| Lymphoma | associated_with | disease | 0.65 |
| Tumor | associated_with | disease | 0.65 |
| Neurodegeneration | activates | disease | 0.65 |
| BAX | expressed_in | gene | 0.60 |
| Igf | therapeutic_target | protein | 0.60 |
| App | therapeutic_target | protein | 0.60 |
| Ras | activates | protein | 0.60 |
| ubiquitin-proteasome | participates_in | pathway | 0.60 |
| Akt | therapeutic_target | pathway | 0.60 |
| MITOCHONDRIA | activates | gene | 0.60 |
| ROS | activates | gene | 0.60 |
| FTLD | activates | gene | 0.60 |
| ALS | activates | gene | 0.60 |
| Unfolded Protein Response | activates | pathway | 0.60 |
| Interneuron | therapeutic_target | cell_type | 0.60 |
| Neuron | therapeutic_target | cell_type | 0.60 |
| SNAP29 | therapeutic_target | gene | 0.60 |
| IGF2BP1 | therapeutic_target | gene | 0.60 |
| MTOR | therapeutic_target | gene | 0.60 |
| GBA1 | interacts_with | gene | 0.60 |
| Source | Relation | Type | Str |
|---|---|---|---|
| AKR1B10 | regulates | gene | 0.80 |
| C1Q | therapeutic_target | gene | 0.60 |
| MTOR | therapeutic_target | gene | 0.60 |
| RAB11 | therapeutic_target | gene | 0.60 |
| STX17 | therapeutic_target | gene | 0.60 |
| RAB5 | therapeutic_target | gene | 0.60 |
| SNAP29 | therapeutic_target | gene | 0.60 |
| IGF2BP1 | therapeutic_target | gene | 0.60 |
| ALS | associated_with | gene | 0.60 |
| INFLAMMATION | activates | gene | 0.60 |
| MITOCHONDRIAL DYSFUNCTION | activates | gene | 0.60 |
| NF-ΚB | activates | gene | 0.60 |
| FTLD | activates | gene | 0.60 |
| NEURODEGENERATION | activates | gene | 0.60 |
| AMYOTROPHIC LATERAL SCLEROSIS | activates | gene | 0.60 |
| ATP | activates | gene | 0.60 |
| MITOCHONDRIA | activates | gene | 0.60 |
| AND | activates | gene | 0.60 |
| ELAVL2 | therapeutic_target | gene | 0.60 |
| METTL3 | therapeutic_target | gene | 0.60 |
| AUTOPHAGY | therapeutic_target | gene | 0.60 |
| FUS | therapeutic_target | gene | 0.60 |
| AKT | therapeutic_target | gene | 0.60 |
| CLPP | causes | gene | 0.60 |
| HTRA2 | causes | gene | 0.60 |
| LYSOSOME | associated_with | gene | 0.60 |
| NEURODEGENERATIVE DISEASES | associated_with | gene | 0.60 |
| ALS | transports | gene | 0.60 |
| NEURODEGENERATION | associated_with | gene | 0.60 |
| COMPLEMENT | associated_with | gene | 0.60 |
| AGING | associated_with | gene | 0.60 |
| FTD | associated_with | entity | 0.60 |
| PI3K | therapeutic_target | gene | 0.60 |
| BDNF | therapeutic_target | gene | 0.60 |
| MTOR | associated_with | gene | 0.60 |
| FUS | associated_with | gene | 0.60 |
| RAB5 | associated_with | gene | 0.60 |
| C1Q/C3 | associated_with | gene | 0.60 |
| RAB11 | associated_with | gene | 0.60 |
| PI3K | associated_with | gene | 0.60 |
| STX17 | associated_with | gene | 0.60 |
| IGF2BP1 | associated_with | gene | 0.60 |
| ELAVL2 | associated_with | gene | 0.60 |
| SNAP29 | associated_with | gene | 0.60 |
| ACSL4 | associated_with | gene | 0.60 |
| BAX | associated_with | gene | 0.60 |
| GPX4 | associated_with | gene | 0.60 |
| HK2 | associated_with | gene | 0.60 |
| CLPP | activates | gene | 0.60 |
| DNM1L | activates | gene | 0.60 |
Hypotheses where this entity is a therapeutic target
| Hypothesis | Score | Disease | Analysis |
|---|---|---|---|
| No targeting hypotheses | |||
Scientific analyses that reference this entity
No analyses mention this entity
Experimental studies targeting or related to this entity
| Experiment | Type | Disease | Score | Feasibility | Model | Status | Est. Cost |
|---|---|---|---|---|---|---|---|
| No experiments found | |||||||
Scientific publications cited in analyses involving this entity
| Title & PMID | Authors | Journal | Year | Citations |
|---|---|---|---|---|
| No papers found | ||||
Multi-agent debates referencing this entity
No debates reference this entity
Hypotheses and analyses mentioning LONP1 in their description or question text
No additional research found