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LONP1 (Gene)

Organism:

Homo sapiens

Taxonomy (Entity type)
Synonyms:CODASS, ENTREZ:9361, HGNC:9479, LON, LONP, LONP1, LonHS, MIM:605490, NM_001276479, NM_001276480, NM_004793, NP_001263408, NP_001263409, NP_004784, NR_076392, PIM1, PRSS15, XM_011528441, XP_011526743, hLON, lon peptidase 1, mitochondrial
Omim:OMIM:605490
Id:75c9b4e3-cb4a-5202-974c-a1daff28582b
Hgnc:HGNC:9479
Entrez:9361
Ensembl:ENSG00000196365
Description:lon peptidase 1, mitochondrial
This gene encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. This protein mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides in the mitochondrial matrix. It may also have a chaperone function in the assembly of inner membrane protein complexes, and participate in the regulation of mitochondrial gene expression and maintenance of the integrity of the mitochondrial genome. Decreased expression of this gene has been noted in a patient with hereditary spastic paraplegia (PMID:18378094). Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2013]

Analyze

Data Distillery
CFDE DD-Knowledge Graph

The CFDE Data Distillery Knowledge Graph contains entities and relationships across the CFDE. View LONP1's neighborhood in the knowledge graph.

Submit
Playbook Workflow Builder
Playbook Workflow Builder: Gene-Centric Workflow

View Gene-Centric information about the gene from a pre-built PWB workflow. View the workflow with LONP1.

Gene Partnership Appyter
CFDE Gene-Centric Appyter

The CFDE Gene Centric Appyter Resolves and Displays Gene-Centric information from CFDE APIs. Execute the Appyter using LONP1.

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Gene and Drug Landing Page Aggregator
GDLPA Landing Pages Links

The Gene and Drug Landing Page Aggregator (GDLPA) finds links to primary and secondary source information from CFDE and other resources. Discover landing pages for LONP1.

Submit
Playbook Workflow Builder
Playbook Workflow Builder

The Playbook Workflow Builder helps you interactively construct workflows leveraging CFDE APIs without code. Start a new workflow with LONP1.

Results found

Linked to

 

Label

Description

 

Gene Set

GSE242555 1 V 0 NICOTINAMIDE HUMAN DN

Gene Set (Entity type)

A Gene Set from LINCS

Gene Set

GSE207871 NPC knockdown S18 1 v 0 human up

Gene Set (Entity type)

A Gene Set from LINCS

Gene Set

GSE128730 3 V 5 NERATINIB HUMAN UP

Gene Set (Entity type)

A Gene Set from LINCS

Gene Set

GSE110178 2 V 3 TRANYLCYPROMINE MOUSE DN

Gene Set (Entity type)

A Gene Set from LINCS

Gene Set

GSE239844 6 V 10 CRIZOTINIB HUMAN DN

Gene Set (Entity type)

A Gene Set from LINCS

Gene Set

GSE229973 3 V 2 CISPLATIN HUMAN DN

Gene Set (Entity type)

A Gene Set from LINCS

Gene Set

GSE152441,GSE152442 6 V 4 FOLIC ACID HUMAN UP

Gene Set (Entity type)

A Gene Set from LINCS

Gene Set

GSE182680,GSE182683 7 V 3 DEXAMETHASONE HUMAN UP

Gene Set (Entity type)

A Gene Set from LINCS

Gene Set

GSE95077 1 V 2 AMILORIDE HUMAN DN

Gene Set (Entity type)

A Gene Set from LINCS

Gene Set

GSE134191 3 V 1 METFORMIN MOUSE DN

Gene Set (Entity type)

A Gene Set from LINCS

  • Gene Set

    GSE242555 1 V 0 NICOTINAMIDE HUMAN DN

    Gene Set (Entity type)

    A Gene Set from LINCS

  • Gene Set

    GSE207871 NPC knockdown S18 1 v 0 human up

    Gene Set (Entity type)

    A Gene Set from LINCS

  • Gene Set

    GSE128730 3 V 5 NERATINIB HUMAN UP

    Gene Set (Entity type)

    A Gene Set from LINCS

  • Gene Set

    GSE110178 2 V 3 TRANYLCYPROMINE MOUSE DN

    Gene Set (Entity type)

    A Gene Set from LINCS

  • Gene Set

    GSE239844 6 V 10 CRIZOTINIB HUMAN DN

    Gene Set (Entity type)

    A Gene Set from LINCS

  • Gene Set

    GSE229973 3 V 2 CISPLATIN HUMAN DN

    Gene Set (Entity type)

    A Gene Set from LINCS

  • Gene Set

    GSE152441,GSE152442 6 V 4 FOLIC ACID HUMAN UP

    Gene Set (Entity type)

    A Gene Set from LINCS

  • Gene Set

    GSE182680,GSE182683 7 V 3 DEXAMETHASONE HUMAN UP

    Gene Set (Entity type)

    A Gene Set from LINCS

  • Gene Set

    GSE95077 1 V 2 AMILORIDE HUMAN DN

    Gene Set (Entity type)

    A Gene Set from LINCS

  • Gene Set

    GSE134191 3 V 1 METFORMIN MOUSE DN

    Gene Set (Entity type)

    A Gene Set from LINCS

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    The CFDE Workbench is actively being developed and maintained by the CFDE Data Resource Center (DRC).The DRC is funded by OT2OD036435 from the Common Fund at the National Institutes of Health.
    Consortium
    About the CFDECF ProgramsCFDE CentersPartnershipsPilot Projects
    Community
    Workbench TutorialTraining & OutreachDocumentationGet QR CodesFunding Opportunities
    Resources
    Data & MetadataToolsChatbotUse CasesPublications
    CFDE Workbench
    Report a bug
    The CFDE Workbench is actively being developed and maintained by the CFDE Data Resource Center (DRC).The DRC is funded by OT2OD036435 from the Common Fund at the National Institutes of Health.
    @CFDE Workbench 2026
    This repository is under review for potential modification in compliance with Administration directives.