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Lonp1 lon peptidase 1, mitochondrial [ Mus musculus (house mouse) ]

Gene ID: 74142, updated on 14-May-2024

Summary

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Official Symbol
Lonp1provided by MGI
Official Full Name
lon peptidase 1, mitochondrialprovided by MGI
Primary source
MGI:MGI:1921392
See related
Ensembl:ENSMUSG00000041168 AllianceGenome:MGI:1921392
Gene type
protein coding
RefSeq status
VALIDATED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as
LON; Prss15; 1200017E13Rik
Summary
Enables ATP hydrolysis activity; ATP-dependent peptidase activity; and DNA binding activity. Acts upstream of or within proteolysis. Located in mitochondrion. Is expressed in several structures, including adrenal medulla; alimentary system; genitourinary system; liver; and lung. Human ortholog(s) of this gene implicated in CODAS syndrome. Orthologous to human LONP1 (lon peptidase 1, mitochondrial). [provided by Alliance of Genome Resources, Apr 2022]
Expression
Broad expression in adrenal adult (RPKM 284.2), ovary adult (RPKM 104.2) and 27 other tissues See more
Orthologs
human all
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Genomic context

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See Lonp1 in Genome Data Viewer
Location:
17 29.42 cM; 17 D
Exon count:
18
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 17 NC_000083.7 (56921297..56933903, complement)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 17 NC_000083.6 (56614297..56626903, complement)

Chromosome 17 - NC_000083.7Genomic Context describing neighboring genes Neighboring gene STARR-positive B cell enhancer ABC_E8687 Neighboring gene mitochondrial contact site and cristae organizing system subunit 13 Neighboring gene ribosomal protein L36 Neighboring gene STARR-positive B cell enhancer ABC_E3210 Neighboring gene cation channel sperm associated auxiliary subunit delta Neighboring gene predicted gene, 33461 Neighboring gene STARR-seq mESC enhancer starr_42897 Neighboring gene RAN binding protein 3 Neighboring gene STARR-seq mESC enhancer starr_42898 Neighboring gene calcyphosine, pseudogene

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

Bibliography

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Related articles in PubMed

  1. Disuse-associated loss of the protease LONP1 in muscle impairs mitochondrial function and causes reduced skeletal muscle mass and strength. Xu Z, et al. Nat Commun, 2022 Feb 16. PMID 35173176, Free PMC Article
  2. Reduced Lon protease 1 expression in podocytes contributes to the pathogenesis of podocytopathy. Gong W, et al. Kidney Int, 2021 Apr. PMID 33181155
  3. Dysfunction of mitochondrial Lon protease and identification of oxidized protein in mouse brain following exposure to MPTP: Implications for Parkinson disease. Bulteau AL, et al. Free Radic Biol Med, 2017 Jul. PMID 28365360
  4. ATP-dependent Lon protease controls tumor bioenergetics by reprogramming mitochondrial activity. Quirós PM, et al. Cell Rep, 2014 Jul 24. PMID 25017063
  5. DNA polymerase gamma mutations that impair holoenzyme stability cause catalytic subunit depletion. Silva-Pinheiro P, et al. Nucleic Acids Res, 2021 May 21. PMID 33956154, Free PMC Article

See all (48) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. LONP1 ameliorates liver injury and improves gluconeogenesis dysfunction in acute-on-chronic liver failure.
    Title: LONP1 ameliorates liver injury and improves gluconeogenesis dysfunction in acute-on-chronic liver failure.
  2. Mitochondrial Lon protease promotes CD4[+] T cell activation by activating the cGAS-STING-TBK1 axis in systemic lupus erythematosus.
    Title: Mitochondrial Lon protease promotes CD4(+) T cell activation by activating the cGAS-STING-TBK1 axis in systemic lupus erythematosus.
  3. Decreased LONP1 expression contributes to DNA damage and meiotic defects in oocytes.
    Title: Decreased LONP1 expression contributes to DNA damage and meiotic defects in oocytes.
  4. LONP1 targets HMGCS2 to protect mitochondrial function and attenuate chronic kidney disease.
    Title: LONP1 targets HMGCS2 to protect mitochondrial function and attenuate chronic kidney disease.
  5. Sirt3 restricts tumor initiation via promoting LONP1 deacetylation and K63 ubiquitination.
    Title: Sirt3 restricts tumor initiation via promoting LONP1 deacetylation and K63 ubiquitination.
  6. LONP1-mediated mitochondrial quality control safeguards metabolic shifts in heart development.
    Title: LONP1-mediated mitochondrial quality control safeguards metabolic shifts in heart development.
  7. The mitochondrial protease LONP1 maintains oocyte development and survival by suppressing nuclear translocation of AIFM1 in mammals.
    Title: The mitochondrial protease LONP1 maintains oocyte development and survival by suppressing nuclear translocation of AIFM1 in mammals.
  8. Disuse-associated loss of the protease LONP1 in muscle impairs mitochondrial function and causes reduced skeletal muscle mass and strength.
    Title: Disuse-associated loss of the protease LONP1 in muscle impairs mitochondrial function and causes reduced skeletal muscle mass and strength.
  9. Mitochondrial LonP1 protease is implicated in the degradation of unstable Parkinson's disease-associated DJ-1/PARK 7 missense mutants.
    Title: Mitochondrial LonP1 protease is implicated in the degradation of unstable Parkinson's disease-associated DJ-1/PARK 7 missense mutants.
  10. Rare and de novo variants in 827 congenital diaphragmatic hernia probands implicate LONP1 as candidate risk gene.
    Title: Rare and de novo variants in 827 congenital diaphragmatic hernia probands implicate LONP1 as candidate risk gene.
Submit: New GeneRIF Correction See all GeneRIFs (21)

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)
  • Endonuclease-mediated (1) 
  • Targeted (6)  1 citation

Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Homology

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables ADP binding ISO
Inferred from Sequence Orthology
more info
  
enables ATP binding ISO
Inferred from Sequence Orthology
more info
  
enables ATP hydrolysis activity IDA
Inferred from Direct Assay
more info
 PubMed 
enables ATP-dependent peptidase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables ATP-dependent peptidase activity IDA
Inferred from Direct Assay
more info
 PubMed 
enables ATP-dependent peptidase activity ISO
Inferred from Sequence Orthology
more info
  
enables DNA binding IEA
Inferred from Electronic Annotation
more info
  
enables DNA polymerase binding ISO
Inferred from Sequence Orthology
more info
  
enables G-quadruplex DNA binding ISO
Inferred from Sequence Orthology
more info
  
enables PH domain binding IPI
Inferred from Physical Interaction
more info
 PubMed 
enables hydrolase activity IEA
Inferred from Electronic Annotation
more info
  
enables identical protein binding ISO
Inferred from Sequence Orthology
more info
  
enables insulin receptor substrate binding IPI
Inferred from Physical Interaction
more info
 PubMed 
NOT enables mitochondrial promoter sequence-specific DNA binding IDA
Inferred from Direct Assay
more info
 PubMed 
enables mitochondrial promoter sequence-specific DNA binding IDA
Inferred from Direct Assay
more info
 PubMed 
enables nucleotide binding IEA
Inferred from Electronic Annotation
more info
  
enables peptidase activity IEA
Inferred from Electronic Annotation
more info
  
enables sequence-specific DNA binding ISO
Inferred from Sequence Orthology
more info
  
enables serine-type endopeptidase activity IEA
Inferred from Electronic Annotation
more info
  
enables serine-type peptidase activity IEA
Inferred from Electronic Annotation
more info
  
enables single-stranded DNA binding IBA
Inferred from Biological aspect of Ancestor
more info
  
enables single-stranded DNA binding IDA
Inferred from Direct Assay
more info
 PubMed 
enables single-stranded RNA binding ISO
Inferred from Sequence Orthology
more info
  
Process Evidence Code Pubs
involved_in cellular response to oxidative stress ISO
Inferred from Sequence Orthology
more info
  
involved_in chaperone-mediated protein complex assembly IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in mitochondrion organization IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in mitochondrion organization ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_or_within negative regulation of insulin receptor signaling pathway IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in oxidation-dependent protein catabolic process ISO
Inferred from Sequence Orthology
more info
  
involved_in protein catabolic process ISO
Inferred from Sequence Orthology
more info
  
involved_in protein quality control for misfolded or incompletely synthesized proteins IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in protein-containing complex assembly ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_or_within proteolysis IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in proteolysis involved in protein catabolic process ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_or_within regulation of peptidyl-tyrosine phosphorylation IDA
Inferred from Direct Assay
more info
 PubMed 
Component Evidence Code Pubs
located_in cytosol ISO
Inferred from Sequence Orthology
more info
  
is_active_in mitochondrial matrix IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in mitochondrial matrix ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrial nucleoid ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrion HDA PubMed 
located_in mitochondrion IDA
Inferred from Direct Assay
more info
 PubMed 
is_active_in mitochondrion ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrion ISO
Inferred from Sequence Orthology
more info
  
located_in nucleoplasm ISO
Inferred from Sequence Orthology
more info
  

General protein information

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Preferred Names
lon protease homolog, mitochondrial
Names
LONP
lon protease-like protein
mitochondrial ATP-dependent protease Lon
protease, serine, 15
serine protease 15
NP_083058.2

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_028782.2 → NP_083058.2  lon protease homolog, mitochondrial precursor

    See identical proteins and their annotated locations for NP_083058.2

    Status: VALIDATED

    Source sequence(s)
    AK160071, BY247676, CK428749
    Consensus CDS
    CCDS28910.1
    UniProtKB/Swiss-Prot
    Q3TSK9, Q3TVL2, Q3TXE4, Q3TZW3, Q8CGK3, Q9DBP9
    UniProtKB/TrEMBL
    Q3V2D0
    Related
    ENSMUSP00000041814.9, ENSMUST00000047226.10
    Conserved Domains (5) summary
    smart00464
    Location:111 → 188
    LON; Found in ATP-dependent protease La (LON)
    TIGR00763
    Location:113 → 936
    lon; endopeptidase La
    pfam00004
    Location:508 → 650
    AAA; ATPase family associated with various cellular activities (AAA)
    cl21455
    Location:478 → 529
    P-loop_NTPase; P-loop containing Nucleoside Triphosphate Hydrolases
    cl21678
    Location:725 → 938
    ChlI; Subunit ChlI of Mg-chelatase

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000083.7 Reference GRCm39 C57BL/6J

    Range
    56921297..56933903 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q8CGK3.2 GenPept UniProtKB/Swiss-Prot:Q8CGK3

Gene LinkOut

The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

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