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    LONP2 lon peptidase 2, peroxisomal [ Homo sapiens (human) ]

    Gene ID: 83752, updated on 10-Dec-2024

    Summary

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    Official Symbol
    LONP2provided by HGNC
    Official Full Name
    lon peptidase 2, peroxisomalprovided by HGNC
    Primary source
    HGNC:HGNC:20598
    See related
    Ensembl:ENSG00000102910 MIM:617774; AllianceGenome:HGNC:20598
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Homo sapiens
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
    Also known as
    LONP; PLON; LONPL; PSLON
    Summary
    In human, peroxisomes function primarily to catalyze fatty acid beta-oxidation and, as a by-product, produce hydrogen peroxide and superoxide. The protein encoded by this gene is an ATP-dependent protease that likely plays a role in maintaining overall peroxisome homeostasis as well as proteolytically degrading peroxisomal proteins damaged by oxidation. The protein has an N-terminal Lon N substrate recognition domain, an ATPase domain, a proteolytic domain, and, in some isoforms, a C-terminal peroxisome targeting sequence. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Jan 2017]
    Expression
    Ubiquitous expression in thyroid (RPKM 23.1), liver (RPKM 21.0) and 25 other tissues See more
    Orthologs
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    Genomic context

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    See LONP2 in Genome Data Viewer
    Location:
    16q12.1
    Exon count:
    19
    Annotation release Status Assembly Chr Location
    RS_2024_08 current GRCh38.p14 (GCF_000001405.40) 16 NC_000016.10 (48244300..48363003)
    RS_2024_08 current T2T-CHM13v2.0 (GCF_009914755.1) 16 NC_060940.1 (54041108..54160261)
    RS_2024_09 previous assembly GRCh37.p13 (GCF_000001405.25) 16 NC_000016.9 (48278211..48396914)

    Chromosome 16 - NC_000016.10Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC124903686 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 10789 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr16:48150856-48151718 Neighboring gene ATP binding cassette subfamily C member 12 Neighboring gene P300/CBP strongly-dependent group 1 enhancer GRCh37_chr16:48156892-48158091 Neighboring gene P300/CBP strongly-dependent group 1 enhancer GRCh37_chr16:48191888-48193087 Neighboring gene H3K27ac hESC enhancer GRCh37_chr16:48198931-48199432 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 7452 Neighboring gene H3K27ac hESC enhancer GRCh37_chr16:48199433-48199932 Neighboring gene Sharpr-MPRA regulatory region 3115 Neighboring gene ATP binding cassette subfamily C member 11 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 10791 Neighboring gene NANOG hESC enhancer GRCh37_chr16:48317173-48317699 Neighboring gene Sharpr-MPRA regulatory region 13528 Neighboring gene H3K27ac hESC enhancer GRCh37_chr16:48353112-48353734 Neighboring gene NANOG hESC enhancer GRCh37_chr16:48370410-48370911 Neighboring gene H3K27ac hESC enhancer GRCh37_chr16:48399426-48400088 Neighboring gene ubiquitin A-52 residue ribosomal protein fusion product 1 pseudogene 8 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr16:48411713-48412214 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 7454 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 7455 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 7456 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr16:48432985-48433730 Neighboring gene siah E3 ubiquitin protein ligase 1 Neighboring gene NANOG-H3K27ac-H3K4me1 hESC enhancer GRCh37_chr16:48450115-48450902 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 10792 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 10793 Neighboring gene OCT4-NANOG hESC enhancer GRCh37_chr16:48465998-48466542 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr16:48489683-48490182 Neighboring gene chromosome 18 open reading frame 32 pseudogene Neighboring gene small nucleolar RNA U13

    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. The peroxisomal Lon protease LonP2 in aging and disease: functions and comparisons with mitochondrial Lon protease LonP1. Pomatto LC, et al. Biol Rev Camb Philos Soc, 2017 May. PMID 26852705, Free PMC Article
    2. Lon Peptidase 2, Peroxisomal (LONP2) Contributes to Cervical Carcinogenesis via Oxidative Stress. Wu W, et al. Med Sci Monit, 2018 Mar 4. PMID 29502128, Free PMC Article
    3. Contribution of peroxisome-specific isoform of Lon protease in sorting PTS1 proteins to peroxisomes. Omi S, et al. J Biochem, 2008 May. PMID 18281296
    4. Identification of Peroxisomal Protein Complexes with PTS Receptors, Pex5 and Pex7, in Mammalian Cells. Okumoto K, et al. Subcell Biochem, 2018. PMID 30378028
    5. Two proteases, trypsin domain-containing 1 (Tysnd1) and peroxisomal lon protease (PsLon), cooperatively regulate fatty acid β-oxidation in peroxisomal matrix. Okumoto K, et al. J Biol Chem, 2011 Dec 30. PMID 22002062, Free PMC Article

    See all (46) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. Findings suggest that LONP2 promotes cervical tumorigenesis via oxidative stress.
      Title: Lon Peptidase 2, Peroxisomal (LONP2) Contributes to Cervical Carcinogenesis via Oxidative Stress.
    2. The proteolytic activity of oligomeric Tysnd1 is in turn controlled by self-cleavage of Tysnd1 and degradation of Tysnd1 cleavage products by PsLon.
      Title: Two proteases, trypsin domain-containing 1 (Tysnd1) and peroxisomal lon protease (PsLon), cooperatively regulate fatty acid β-oxidation in peroxisomal matrix.
    Submit: New GeneRIF Correction

    Phenotypes

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    Find tests for this gene in the NIH Genetic Testing Registry (GTR)

    Review eQTL and phenotype association data in this region using PheGenI

    Variation

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    See variants in ClinVar

    See studies and variants in dbVar

    See Variation Viewer (GRCh37.p13)

    See Variation Viewer (GRCh38)

    Interactions

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    Products Interactant Other Gene Complex Source Pubs Description

    General gene information

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    Markers

    Homology

    Clone Names

    • MGC4840, FLJ31489

    Gene Ontology Provided by GOA

    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 IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables enzyme binding IPI
    Inferred from Physical Interaction
    more info
    		 PubMed 
    enables peptidase activity IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    enables protease binding IPI
    Inferred from Physical Interaction
    more info
    		 PubMed 
    enables protein binding IPI
    Inferred from Physical Interaction
    more info
    		 PubMed 
    enables serine-type endopeptidase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    Process Evidence Code Pubs
    involved_in peroxisome organization NAS
    Non-traceable Author Statement
    more info
    		 PubMed 
    involved_in protein import into peroxisome matrix IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in protein processing IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in protein processing IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in protein quality control for misfolded or incompletely synthesized proteins IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in protein targeting to peroxisome IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in protein targeting to peroxisome IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in regulation of fatty acid beta-oxidation IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in response to organic cyclic compound IEA
    Inferred from Electronic Annotation
    more info
    		  
    Component Evidence Code Pubs
    located_in cytosol TAS
    Traceable Author Statement
    more info
    		  
    located_in membrane HDA PubMed 
    located_in nucleus IEA
    Inferred from Electronic Annotation
    more info
    		  
    is_active_in peroxisomal matrix IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    located_in peroxisomal matrix TAS
    Traceable Author Statement
    more info
    		  
    located_in peroxisome IDA
    Inferred from Direct Assay
    more info
    		 PubMed 

    General protein information

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    Preferred Names
    lon protease homolog 2, peroxisomal
    Names
    lon protease 2
    lon protease-like protein 2
    peroxisomal LON protease like
    peroxisomal Lon protease homolog 2
    NP_001287877.1
    NP_001335007.1
    NP_113678.2
    XP_016879244.1
    XP_016879245.1
    XP_047290693.1
    XP_047290694.1
    XP_054170079.1
    XP_054170080.1
    XP_054170081.1
    XP_054170082.1

    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.

    Genomic

    1. NG_053011.1 RefSeqGene

      Range
      5134..118183
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_001300948.3 → NP_001287877.1  lon protease homolog 2, peroxisomal isoform 2

      See identical proteins and their annotated locations for NP_001287877.1

      Status: REVIEWED

      Description
      Transcript Variant: This variant (2) lacks an alternate in-frame exon in the 5' coding region compared to variant 1. It encodes isoform 2 which is shorter than isoform 1.
      Source sequence(s)
      AC007600, AC023818, BC143246, BX104260
      Consensus CDS
      CCDS73880.1
      UniProtKB/Swiss-Prot
      Q86WA8
      Related
      ENSP00000445426.1, ENST00000535754.5
      Conserved Domains (4) summary
      COG0466
      Location:11 → 794
      Lon; ATP-dependent Lon protease, bacterial type [Posttranslational modification, protein turnover, chaperones]
      cl19481
      Location:4 → 193
      LON; Found in ATP-dependent protease La (LON)
      pfam00004
      Location:327 → 469
      AAA; ATPase family associated with various cellular activities (AAA)
      cl21678
      Location:590 → 793
      ChlI; Subunit ChlI of Mg-chelatase

    2. NM_001348078.2 → NP_001335007.1  lon protease homolog 2, peroxisomal isoform 3

      Status: REVIEWED

      Description
      Transcript Variant: This variant (3) has multiple differences at its 3' terminus, compared to variant 1, which result in a novel 3' coding region and 3' UTR. It encodes isoform 3 which is longer than isoform 1 and lacks the C-terminal peroximsome targeting sequence present in isoforms 1 and 2.
      Source sequence(s)
      AC007600, AC023818, CA427778
      Conserved Domains (1) summary
      cl26147
      Location:11 → 838
      Lon; ATP-dependent Lon protease, bacterial type [Posttranslational modification, protein turnover, chaperones]

    3. NM_031490.5 → NP_113678.2  lon protease homolog 2, peroxisomal isoform 1

      See identical proteins and their annotated locations for NP_113678.2

      Status: REVIEWED

      Description
      Transcript Variant: This variant (1) represents the longest transcript and encodes isoform (1).
      Source sequence(s)
      AC023818, AJ548761, BX104260
      Consensus CDS
      CCDS10734.1
      UniProtKB/Swiss-Prot
      B7ZKL7, Q0D2H6, Q86WA8, Q8N3B9, Q8NCE9, Q96K43
      Related
      ENSP00000285737.4, ENST00000285737.9
      Conserved Domains (4) summary
      COG0466
      Location:11 → 838
      Lon; ATP-dependent Lon protease, bacterial type [Posttranslational modification, protein turnover, chaperones]
      cl19481
      Location:4 → 144
      LON; Found in ATP-dependent protease La (LON)
      pfam00004
      Location:371 → 513
      AAA; ATPase family associated with various cellular activities (AAA)
      cl21678
      Location:634 → 837
      ChlI; Subunit ChlI of Mg-chelatase

    RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2024_08

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

    Reference GRCh38.p14 Primary Assembly

    Genomic

    1. NC_000016.10 Reference GRCh38.p14 Primary Assembly

      Range
      48244300..48363003
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_047434738.1 → XP_047290694.1  lon protease homolog 2, peroxisomal isoform X3

    2. XM_047434737.1 → XP_047290693.1  lon protease homolog 2, peroxisomal isoform X1

      UniProtKB/TrEMBL
      B4DGU5

    3. XM_017023756.2 → XP_016879245.1  lon protease homolog 2, peroxisomal isoform X2

      UniProtKB/TrEMBL
      B4DGU5, E7EN44
      Related
      ENSP00000415983.3, ENST00000416006.7
      Conserved Domains (1) summary
      cl26147
      Location:11 → 541
      Lon; ATP-dependent Lon protease, bacterial type [Posttranslational modification, protein turnover, chaperones]

    4. XM_017023755.3 → XP_016879244.1  lon protease homolog 2, peroxisomal isoform X1

      UniProtKB/TrEMBL
      B4DGU5

    Alternate T2T-CHM13v2.0

    Genomic

    1. NC_060940.1 Alternate T2T-CHM13v2.0

      Range
      54041108..54160261
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_054314107.1 → XP_054170082.1  lon protease homolog 2, peroxisomal isoform X3

    2. XM_054314104.1 → XP_054170079.1  lon protease homolog 2, peroxisomal isoform X1

      UniProtKB/TrEMBL
      B4DGU5

    3. XM_054314106.1 → XP_054170081.1  lon protease homolog 2, peroxisomal isoform X2

      UniProtKB/TrEMBL
      B4DGU5, E7EN44

    4. XM_054314105.1 → XP_054170080.1  lon protease homolog 2, peroxisomal isoform X1

      UniProtKB/TrEMBL
      B4DGU5

    Suppressed Reference Sequence(s)

    The following Reference Sequences have been suppressed. Explain

    1. NR_040677.1: Suppressed sequence

      Description
      NR_040677.1: This RefSeq was removed because the gene was discontinued.
    Nucleotide Protein
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q86WA8.1 GenPept UniProtKB/Swiss-Prot:Q86WA8

    Gene LinkOut

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

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