gene

LONP1

Entity Detail — Knowledge Graph Node

Understanding Entity Pages

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.

314Connections

0Hypotheses

0Analyses

50Outgoing

50Incoming

Summary

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 Info

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, Ftd, Lymphoma, Neurodegeneration, Tumor
Interactions	CEBPB, SPG7, MAP1LC3A, DDIT3, JUN, TIMM50
KG Connections	314 knowledge graph edges
Databases	GeneCards NCBI Gene HPA STRING

Wiki Pages (17)

Knowledge base pages for this entity

Canonical Page

Pathway Diagram

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

Outgoing (196)

Target	Relation	Type	Str
Mitochondrial Proteostasis	involved_in	process	0.85
mitochondrial proteostasis	regulates	process	0.85
Proteostasis	involved_in	process	0.85
AKR1B10	downstream_of	gene	0.80
Als	activates	disease	0.75
Neurodegeneration	therapeutic_target	disease	0.65
Aging	therapeutic_target	disease	0.65
Ms	therapeutic_target	disease	0.65
Als	associated_with	disease	0.65
Aging	associated_with	disease	0.65
Ftd	associated_with	disease	0.65
Neurodegeneration	associated_with	disease	0.65
Carcinoma	expressed_in	disease	0.65
Ms	expressed_in	disease	0.65
Tumor	expressed_in	disease	0.65
Heart Failure	inhibits	disease	0.65
Ms	inhibits	disease	0.65
ALS	transports	disease	0.65
Aging	activates	disease	0.65
Inflammation	activates	disease	0.65
Tumor	associated_with	disease	0.65
Lymphoma	associated_with	disease	0.65
Parkinson	interacts_with	disease	0.65
Als	interacts_with	disease	0.65
Neurodegeneration	interacts_with	disease	0.65
Fibrosis	activates	disease	0.65
Metabolic Syndrome	activates	disease	0.65
Hepatitis	activates	disease	0.65
Amyotrophic Lateral Sclerosis	activates	disease	0.65
Neurodegeneration	activates	disease	0.65
Aging	encodes	disease	0.65
Neurodegeneration	encodes	disease	0.65
Als	transports	disease	0.65
BDNF	therapeutic_target	gene	0.60
FUS	therapeutic_target	gene	0.60
C1Q	therapeutic_target	gene	0.60
AKT	therapeutic_target	gene	0.60
BAX	expressed_in	gene	0.60
ELAVL2	therapeutic_target	gene	0.60
RAB5	therapeutic_target	gene	0.60
STX17	therapeutic_target	gene	0.60
CLPP	associated_with	gene	0.60
RAB11	therapeutic_target	gene	0.60
CEBPB	inhibits	gene	0.60
SPG7	inhibits	gene	0.60
MAP1LC3A	inhibits	gene	0.60
DDIT3	inhibits	gene	0.60
JUN	inhibits	gene	0.60
TIMM50	inhibits	gene	0.60
HSPA9	inhibits	gene	0.60

Incoming (118)

Source	Relation	Type	Str
AKR1B10	regulates	gene	0.80
ELAVL2	therapeutic_target	gene	0.60
C1Q	therapeutic_target	gene	0.60
MTOR	therapeutic_target	gene	0.60
CLPP	associated_with	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
PI3K	therapeutic_target	gene	0.60
BDNF	therapeutic_target	gene	0.60
AKT	associated_with	gene	0.60
BDNF	associated_with	gene	0.60
FUS	therapeutic_target	gene	0.60
AKT	therapeutic_target	gene	0.60
BNIP3	inhibits	gene	0.60
VDAC1	inhibits	gene	0.60
FUNDC1	inhibits	gene	0.60
TOMM20	inhibits	gene	0.60
TIMM9	inhibits	gene	0.60
TIMM17B	inhibits	gene	0.60
OMA1	inhibits	gene	0.60
TIMM23	inhibits	gene	0.60
MTOR	inhibits	gene	0.60
NRF1	inhibits	gene	0.60
HSPD1	inhibits	gene	0.60
MFN2	inhibits	gene	0.60
MFF	inhibits	gene	0.60
BECN1	inhibits	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
C1Q	associated_with	gene	0.60
SDHA	expressed_in	gene	0.60
MFN2	activates	gene	0.60
BDNF	activates	gene	0.60
SOD2	activates	gene	0.60
NRF2	activates	gene	0.60
FNDC5	activates	gene	0.60
FOXO1	activates	gene	0.60
FIS1	activates	gene	0.60
OGG1	activates	gene	0.60

Targeting Hypotheses (0)

Hypotheses where this entity is a therapeutic target

Hypothesis	Score	Disease	Analysis
No targeting hypotheses

Mentioning Analyses (0)

Scientific analyses that reference this entity

No analyses mention this entity

Related Papers (0)

Scientific publications cited in analyses involving this entity

Title & PMID	Authors	Journal	Year	Citations
No papers found

LONP1

Understanding Entity Pages

Summary

Wiki Pages (17)

LONP1

Mitochondrial Quality Control

Mitochondrial Dysfunction in Parkinson's Disease

Mitophagy Gate Therapy: PINK1/Parkin + TFEB Priming

COA8 Gene - Cytochrome c Oxidase Assembly Factor 8

SUCLA2 Gene

TIMM23 — Translocase of Inner Mitochondrial Membrane 23

Mitochondrial Quality Control Network Pathway

CLPP Protein

SPATA5 Protein

Twinkle (Mitochondrial DNA Helicase)

CLPP (Caseinolytic Mitochondrial Matrix Peptidase Proteolytic)

OGG1 Gene

Hsp10 Protein

YME1L1 Protein

HSPD2 Gene

Genes

Pathway Diagram

Outgoing (196)

Incoming (118)

Targeting Hypotheses (0)

Mentioning Analyses (0)

Related Papers (0)