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    Lon Lon protease [ Drosophila melanogaster (fruit fly) ]

    Gene ID: 40138, updated on 27-Dec-2024

    Summary

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    Official Symbol
    Lonprovided by FlyBase
    Official Full Name
    Lon proteaseprovided by FlyBase
    Primary source
    FLYBASE:FBgn0036892
    Locus tag
    Dmel_CG8798
    See related
    AllianceGenome:FB:FBgn0036892
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Drosophila melanogaster
    Lineage
    Eukaryota; Metazoa; Ecdysozoa; Arthropoda; Hexapoda; Insecta; Pterygota; Neoptera; Endopterygota; Diptera; Brachycera; Muscomorpha; Ephydroidea; Drosophilidae; Drosophila; Sophophora
    Also known as
    anon-WO0118547.350; CG8798; dLon P1; Dmel\CG8798; LON; LONP; LONP1
    Summary
    Enables serine hydrolase activity. Involved in mitochondrion organization and positive regulation of protein catabolic process. Located in mitochondrion. Is expressed in several structures, including Malpighian tubule primordium; adult head; adult heart; embryonic/larval midgut primordium; and gut section. Used to study CODAS syndrome. Human ortholog(s) of this gene implicated in CODAS syndrome. Orthologous to human LONP1 (lon peptidase 1, mitochondrial). [provided by Alliance of Genome Resources, Dec 2024]
    Orthologs
    all
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    Genomic context

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    See Lon in Genome Data Viewer
    Location:
    76B9-76B9; 3-45 cM
    Exon count:
    9
    Annotation release Status Assembly Chr Location
    Release 6.54 current Release 6 plus ISO1 MT (GCF_000001215.4) 3L NT_037436.4 (19607857..19612199, complement)
    Release 5.57 previous assembly Release 5 (GCF_000001215.2) 3L NT_037436.3 (19600957..19605299, complement)

    Chromosome 3L - NT_037436.4Genomic Context describing neighboring genes Neighboring gene uncharacterized protein Neighboring gene lush Neighboring gene uncharacterized protein Neighboring gene lethal (3) 76BDm Neighboring gene Phosphatidylinositol glycan anchor biosynthesis class F Neighboring gene anti-silencing factor 1

    Genomic regions, transcripts, and products

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    Go to nucleotide: Graphics FASTA GenBank

    Bibliography

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    Related articles in PubMed

    1. Inactivation of Lon protease reveals a link between mitochondrial unfolded protein stress and mitochondrial translation inhibition. Pareek G, et al. Cell Death Dis, 2018 Dec 5. PMID 30518747, Free PMC Article
    2. The Mitochondrial Lon Protease Is Required for Age-Specific and Sex-Specific Adaptation to Oxidative Stress. Pomatto LCD, et al. Curr Biol, 2017 Jan 9. PMID 27916526, Free PMC Article
    3. Protein-Protein Interactions of Seryl-tRNA Synthetases with Emphasis on Human Counterparts and Their Connection to Health and Disease. Dulic M, et al. Life (Basel), 2024 Jan 15. PMID 38255739, Free PMC Article
    4. Identification of Moonlighting Proteins in Genomes Using Text Mining Techniques. Jain A, et al. Proteomics, 2018 Nov. PMID 30260564, Free PMC Article
    5. Autophagy accounts for approximately one-third of mitochondrial protein turnover and is protein selective. Vincow ES, et al. Autophagy, 2019 Sep. PMID 30865561, Free PMC Article

    See all (65) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. Inactivation of Lon protease reveals a link between mitochondrial unfolded protein stress and mitochondrial translation inhibition.
      Title: Inactivation of Lon protease reveals a link between mitochondrial unfolded protein stress and mitochondrial translation inhibition.
    2. Lon regulates mitochondrial transcription by stabilizing the mitochondrial TFAMmtDNA ratio via selective degradation of TFAM.
      Title: Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM).
    Submit: New GeneRIF Correction

    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 FlyBase

    Function Evidence Code Pubs
    enables ATP binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables ATP hydrolysis activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables ATP-dependent peptidase activity IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables ATP-dependent peptidase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables serine hydrolase activity HDA PubMed 
    enables serine-type endopeptidase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables single-stranded DNA binding IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    Process Evidence Code Pubs
    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 IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in positive regulation of protein catabolic process IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in protein catabolic process IEA
    Inferred from Electronic Annotation
    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 quality control for misfolded or incompletely synthesized proteins IEA
    Inferred from Electronic Annotation
    more info
    		  
    Component Evidence Code Pubs
    is_active_in mitochondrial matrix IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    located_in mitochondrion IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    located_in peroxisome ISS
    Inferred from Sequence or Structural Similarity
    more info
    		 PubMed 

    General protein information

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    Preferred Names
    Lon protease
    Names
    CG8798-PA
    CG8798-PC
    Lon-PA
    Lon-PC
    NP_649133.1
    NP_730435.2

    NCBI Reference Sequences (RefSeq)

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    Genome Annotation

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

    Reference assembly

    Genomic

    1. NT_037436.4 Reference assembly

      Range
      19607857..19612199 complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_140876.3 → NP_649133.1  Lon protease, isoform A [Drosophila melanogaster]

      See identical proteins and their annotated locations for NP_649133.1

      Status: REVIEWED

      UniProtKB/TrEMBL
      Q960X9
      Related
      FBpp0074735
      Conserved Domains (5) summary
      smart00464
      Location:93 → 148
      LON; Found in ATP-dependent protease La (LON)
      TIGR00763
      Location:95 → 972
      lon; endopeptidase La
      pfam00004
      Location:542 → 684
      AAA; ATPase family associated with various cellular activities (AAA)
      cl21455
      Location:512 → 563
      P-loop_NTPase; P-loop containing Nucleoside Triphosphate Hydrolases
      cl21678
      Location:760 → 973
      ChlI; Subunit ChlI of Mg-chelatase

    2. NM_168806.3 → NP_730435.2  Lon protease, isoform C [Drosophila melanogaster]

      See identical proteins and their annotated locations for NP_730435.2

      Status: REVIEWED

      UniProtKB/Swiss-Prot
      Q7KUT2, Q9VW20
      UniProtKB/TrEMBL
      Q960X9
      Conserved Domains (5) summary
      smart00464
      Location:93 → 148
      LON; Found in ATP-dependent protease La (LON)
      TIGR00763
      Location:95 → 990
      lon; endopeptidase La
      pfam00004
      Location:560 → 702
      AAA; ATPase family associated with various cellular activities (AAA)
      cl21455
      Location:530 → 581
      P-loop_NTPase; P-loop containing Nucleoside Triphosphate Hydrolases
      cl21678
      Location:778 → 991
      ChlI; Subunit ChlI of Mg-chelatase
    Nucleotide Protein
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q7KUT2.1 Q7KUT2
    Q960X9 Q960X9

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