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Protein

Lon protease homolog 2, peroxisomal

Gene

LONP2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

ATP-dependent serine protease that mediates the selective degradation of misfolded and unassembled polypeptides in the peroxisomal matrix. Necessary for type 2 peroxisome targeting signal (PTS2)-containing protein processing and facilitates peroxisome matrix protein import (By similarity). May indirectly regulate peroxisomal fatty acid beta-oxidation through degradation of the self-processed forms of TYSND1.UniRule annotation1 Publication

Catalytic activityi

Hydrolysis of proteins in presence of ATP.UniRule annotation

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei743UniRule annotation1
Active sitei786UniRule annotation1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi375 – 382ATPUniRule annotation8

GO - Molecular functioni

  • ATP binding Source: UniProtKB-KW
  • ATP-dependent peptidase activity Source: InterPro
  • enzyme binding Source: UniProtKB
  • peptidase activity Source: UniProtKB
  • protease binding Source: UniProtKB
  • serine-type endopeptidase activity Source: InterPro
  • signaling receptor binding Source: UniProtKB

GO - Biological processi

  • peroxisome organization Source: UniProtKB
  • protein processing Source: UniProtKB
  • protein quality control for misfolded or incompletely synthesized proteins Source: InterPro
  • protein targeting to peroxisome Source: UniProtKB
  • regulation of fatty acid beta-oxidation Source: UniProtKB
  • response to organic cyclic compound Source: Ensembl

Keywordsi

Molecular functionHydrolase, Protease, Serine protease
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

ReactomeiR-HSA-390471 Association of TriC/CCT with target proteins during biosynthesis
R-HSA-9033241 Peroxisomal protein import

Protein family/group databases

MEROPSiS16.006

Names & Taxonomyi

Protein namesi
Recommended name:
Lon protease homolog 2, peroxisomalUniRule annotation (EC:3.4.21.53UniRule annotation)
Alternative name(s):
Lon protease-like protein 2UniRule annotation
Short name:
Lon protease 2UniRule annotation
Peroxisomal Lon proteaseUniRule annotation
Short name:
pLon1 Publication
Gene namesi
Name:LONP2UniRule annotation
Synonyms:LONP
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 16

Organism-specific databases

EuPathDBiHostDB:ENSG00000102910.13
HGNCiHGNC:20598 LONP2
MIMi617774 gene
neXtProtiNX_Q86WA8

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Peroxisome

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi743S → A: Reduces degradation of self-processed forms of TYSND1. Loss of ACOX1-processing. 2 Publications1
Mutagenesisi850 – 852Missing : Loss of peroxisomal localization. 1 Publication3

Organism-specific databases

OpenTargetsiENSG00000102910
PharmGKBiPA162394186

Polymorphism and mutation databases

BioMutaiLONP2
DMDMi74727668

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00002876402 – 852Lon protease homolog 2, peroxisomalAdd BLAST851

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylserineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ86WA8
MaxQBiQ86WA8
PaxDbiQ86WA8
PeptideAtlasiQ86WA8
PRIDEiQ86WA8
ProteomicsDBi70138

PTM databases

iPTMnetiQ86WA8
PhosphoSitePlusiQ86WA8

Expressioni

Tissue specificityi

Widely expressed, with high levels in the liver, kidney and pancreas.1 Publication

Gene expression databases

BgeeiENSG00000102910
CleanExiHS_LONP2
ExpressionAtlasiQ86WA8 baseline and differential
GenevisibleiQ86WA8 HS

Organism-specific databases

HPAiHPA008862

Interactioni

Subunit structurei

Interacts with PEX5 (By similarity) (PubMed:22002062). Interacts with TYSND1 (By similarity) (PubMed:22002062). May interact with enzymes involved in beta-oxidation of fatty acids, including ACOX1/AOX (PubMed:18281296).UniRule annotation2 Publications

GO - Molecular functioni

  • enzyme binding Source: UniProtKB
  • protease binding Source: UniProtKB
  • signaling receptor binding Source: UniProtKB

Protein-protein interaction databases

BioGridi123755, 30 interactors
IntActiQ86WA8, 10 interactors
STRINGi9606.ENSP00000285737

Structurei

3D structure databases

ProteinModelPortaliQ86WA8
SMRiQ86WA8
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini13 – 220Lon N-terminalPROSITE-ProRule annotationAdd BLAST208
Domaini651 – 837Lon proteolyticPROSITE-ProRule annotationAdd BLAST187

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi850 – 852Microbody targeting signalUniRule annotation3

Sequence similaritiesi

Belongs to the peptidase S16 family.UniRule annotation

Phylogenomic databases

eggNOGiKOG2004 Eukaryota
COG0466 LUCA
GeneTreeiENSGT00530000063553
HOGENOMiHOG000261408
HOVERGENiHBG000798
InParanoidiQ86WA8
KOiK01338
OMAiPILCLYG
OrthoDBiEOG091G02SC
PhylomeDBiQ86WA8
TreeFamiTF317215

Family and domain databases

Gene3Di3.30.230.10, 1 hit
HAMAPiMF_03121 lonp2_euk, 1 hit
InterProiView protein in InterPro
IPR003593 AAA+_ATPase
IPR003959 ATPase_AAA_core
IPR004815 Lon_bac/euk-typ
IPR008269 Lon_proteolytic
IPR027065 Lon_Prtase
IPR003111 Lon_substr-bd
IPR027501 Lonp2_euk
IPR027417 P-loop_NTPase
IPR008268 Peptidase_S16_AS
IPR015947 PUA-like_sf
IPR020568 Ribosomal_S5_D2-typ_fold
IPR014721 Ribosomal_S5_D2-typ_fold_subgr
PANTHERiPTHR10046 PTHR10046, 1 hit
PfamiView protein in Pfam
PF00004 AAA, 1 hit
PF05362 Lon_C, 1 hit
PF02190 LON_substr_bdg, 1 hit
PIRSFiPIRSF001174 Lon_proteas, 1 hit
SMARTiView protein in SMART
SM00382 AAA, 1 hit
SM00464 LON, 1 hit
SUPFAMiSSF52540 SSF52540, 1 hit
SSF54211 SSF54211, 1 hit
SSF88697 SSF88697, 1 hit
TIGRFAMsiTIGR00763 lon, 1 hit
PROSITEiView protein in PROSITE
PS51787 LON_N, 1 hit
PS51786 LON_PROTEOLYTIC, 1 hit
PS01046 LON_SER, 1 hit

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q86WA8-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MSSVSPIQIP SRLPLLLTHE GVLLPGSTMR TSVDSARNLQ LVRSRLLKGT
60 70 80 90 100
SLQSTILGVI PNTPDPASDA QDLPPLHRIG TAALAVQVVG SNWPKPHYTL
110 120 130 140 150
LITGLCRFQI VQVLKEKPYP IAEVEQLDRL EEFPNTCKMR EELGELSEQF
160 170 180 190 200
YKYAVQLVEM LDMSVPAVAK LRRLLDSLPR EALPDILTSI IRTSNKEKLQ
210 220 230 240 250
ILDAVSLEER FKMTIPLLVR QIEGLKLLQK TRKPKQDDDK RVIAIRPIRR
260 270 280 290 300
ITHISGTLED EDEDEDNDDI VMLEKKIRTS SMPEQAHKVC VKEIKRLKKM
310 320 330 340 350
PQSMPEYALT RNYLELMVEL PWNKSTTDRL DIRAARILLD NDHYAMEKLK
360 370 380 390 400
KRVLEYLAVR QLKNNLKGPI LCFVGPPGVG KTSVGRSVAK TLGREFHRIA
410 420 430 440 450
LGGVCDQSDI RGHRRTYVGS MPGRIINGLK TVGVNNPVFL LDEVDKLGKS
460 470 480 490 500
LQGDPAAALL EVLDPEQNHN FTDHYLNVAF DLSQVLFIAT ANTTATIPAA
510 520 530 540 550
LLDRMEIIQV PGYTQEEKIE IAHRHLIPKQ LEQHGLTPQQ IQIPQVTTLD
560 570 580 590 600
IITRYTREAG VRSLDRKLGA ICRAVAVKVA EGQHKEAKLD RSDVTEREGC
610 620 630 640 650
REHILEDEKP ESISDTTDLA LPPEMPILID FHALKDILGP PMYEMEVSQR
660 670 680 690 700
LSQPGVAIGL AWTPLGGEIM FVEASRMDGE GQLTLTGQLG DVMKESAHLA
710 720 730 740 750
ISWLRSNAKK YQLTNAFGSF DLLDNTDIHL HFPAGAVTKD GPSAGVTIVT
760 770 780 790 800
CLASLFSGRL VRSDVAMTGE ITLRGLVLPV GGIKDKVLAA HRAGLKQVII
810 820 830 840 850
PRRNEKDLEG IPGNVRQDLS FVTASCLDEV LNAAFDGGFT VKTRPGLLNS

KL
Length:852
Mass (Da):94,617
Last modified:June 1, 2003 - v1
Checksum:i00444E4FF47417AC
GO
Isoform 2 (identifier: Q86WA8-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     156-199: Missing.

Note: No experimental confirmation available.
Show »
Length:808
Mass (Da):89,660
Checksum:iA3BF87CB9C823DA1
GO

Sequence cautioni

The sequence BAB55278 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti255S → A in AAI10435 (PubMed:15489334).Curated1
Sequence conflicti628L → F in BAC11201 (PubMed:14702039).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_056190156 – 199Missing in isoform 2. 1 PublicationAdd BLAST44

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ548761 mRNA Translation: CAD68987.1
AK027666 mRNA Translation: BAB55278.1 Different initiation.
AK074775 mRNA Translation: BAC11201.1
AC007600 Genomic DNA No translation available.
AC023818 Genomic DNA No translation available.
BC093910 mRNA Translation: AAH93910.1
BC093912 mRNA Translation: AAH93912.1
BC110434 mRNA Translation: AAI10435.1
BC143246 mRNA Translation: AAI43247.1
AL834201 mRNA Translation: CAD38889.1
CCDSiCCDS10734.1 [Q86WA8-1]
CCDS73880.1 [Q86WA8-2]
RefSeqiNP_001287877.1, NM_001300948.2 [Q86WA8-2]
NP_113678.2, NM_031490.4 [Q86WA8-1]
UniGeneiHs.295923
Hs.652267
Hs.731827
Hs.744137

Genome annotation databases

EnsembliENST00000285737; ENSP00000285737; ENSG00000102910 [Q86WA8-1]
ENST00000535754; ENSP00000445426; ENSG00000102910 [Q86WA8-2]
GeneIDi83752
KEGGihsa:83752
UCSCiuc002efi.2 human [Q86WA8-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiLONP2_HUMAN
AccessioniPrimary (citable) accession number: Q86WA8
Secondary accession number(s): B7ZKL7
, Q0D2H6, Q8N3B9, Q8NCE9, Q96K43
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 15, 2007
Last sequence update: June 1, 2003
Last modified: June 20, 2018
This is version 144 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 16
    Human chromosome 16: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. Peptidase families
    Classification of peptidase families and list of entries
  4. SIMILARITY comments
    Index of protein domains and families

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