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

Gene ID: 83752, updated on 3-Apr-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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Location:
16q12.1
Exon count:
19
Annotation release Status Assembly Chr Location
RS_2023_10 current GRCh38.p14 (GCF_000001405.40) 16 NC_000016.10 (48244300..48363003)
RS_2023_10 current T2T-CHM13v2.0 (GCF_009914755.1) 16 NC_060940.1 (54041108..54160261)
105.20220307 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

Expression

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  • Project title: HPA RNA-seq normal tissues
  • Description: RNA-seq was performed of tissue samples from 95 human individuals representing 27 different tissues in order to determine tissue-specificity of all protein-coding genes
  • BioProject: PRJEB4337
  • Publication: PMID 24309898
  • Analysis date: Wed Apr 4 07:08:55 2018

Bibliography

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Related articles in PubMed

  1. 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
  2. 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
  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 (42) 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)

Genotypes

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_2023_10

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

  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

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

    UniProtKB/TrEMBL
    E7EN44

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

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

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