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

Gene ID: 83752, updated on 7-Jun-2020

Summary

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
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

Genomic context

See LONP2 in Genome Data Viewer
Location:
16q12.1
Exon count:
18
Annotation release Status Assembly Chr Location
109.20200522 current GRCh38.p13 (GCF_000001405.39) 16 NC_000016.10 (48244300..48363003)
105 previous assembly GRCh37.p13 (GCF_000001405.25) 16 NC_000016.9 (48278211..48387888)

Chromosome 16 - NC_000016.10Genomic Context describing neighboring genes Neighboring gene long intergenic non-protein coding RNA 2134 Neighboring gene ATP binding cassette subfamily C member 12 Neighboring gene ATP binding cassette subfamily C member 11 Neighboring gene Sharpr-MPRA regulatory region 13528 Neighboring gene ubiquitin A-52 residue ribosomal protein fusion product 1 pseudogene 8 Neighboring gene siah E3 ubiquitin protein ligase 1 Neighboring gene chromosome 18 open reading frame 32 pseudogene Neighboring gene molybdenum cofactor synthesis 1 pseudogene 1

Genomic regions, transcripts, and products

Expression

  • 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

GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Interactions

Products Interactant Other Gene Complex Source Pubs Description

General gene information

Markers

Homology

Clone Names

  • MGC4840, FLJ31489

Gene Ontology Provided by GOA

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

General protein information

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

NCBI Reference Sequences (RefSeq)

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..123837
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_001300948.3NP_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:11794
    Lon; ATP-dependent Lon protease, bacterial type [Posttranslational modification, protein turnover, chaperones]
    cl19481
    Location:4193
    LON; Found in ATP-dependent protease La (LON)
    pfam00004
    Location:327469
    AAA; ATPase family associated with various cellular activities (AAA)
    cl21678
    Location:590793
    ChlI; Subunit ChlI of Mg-chelatase
  2. NM_001348078.2NP_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:11838
    Lon; ATP-dependent Lon protease, bacterial type [Posttranslational modification, protein turnover, chaperones]
  3. NM_031490.5NP_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
    Q86WA8
    Related
    ENSP00000285737.4, ENST00000285737.9
    Conserved Domains (4) summary
    COG0466
    Location:11838
    Lon; ATP-dependent Lon protease, bacterial type [Posttranslational modification, protein turnover, chaperones]
    cl19481
    Location:4144
    LON; Found in ATP-dependent protease La (LON)
    pfam00004
    Location:371513
    AAA; ATPase family associated with various cellular activities (AAA)
    cl21678
    Location:634837
    ChlI; Subunit ChlI of Mg-chelatase

RefSeqs of Annotated Genomes: Homo sapiens Updated Annotation Release 109.

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

Reference GRCh38.p13 Primary Assembly

Genomic

  1. NC_000016.10 Reference GRCh38.p13 Primary Assembly

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

mRNA and Protein(s)

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

    UniProtKB/TrEMBL
    E7EN44
    Related
    ENSP00000415983.3, ENST00000416006.7
    Conserved Domains (1) summary
    cl26147
    Location:11541
    Lon; ATP-dependent Lon protease, bacterial type [Posttranslational modification, protein turnover, chaperones]
  2. XM_017023755.2XP_016879244.1  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.
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