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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

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