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Protein

Lon protease homolog, mitochondrial

Gene

Lonp1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single-stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site-specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters.UniRule annotation1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei844UniRule annotation1
Active sitei887UniRule annotation1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi512 – 519ATPUniRule annotation8

GO - Molecular functioni

  • ADP binding Source: MGI
  • ATPase activity Source: MGI
  • ATP binding Source: MGI
  • ATP-dependent peptidase activity Source: MGI
  • DNA polymerase binding Source: MGI
  • G-quadruplex DNA binding Source: MGI
  • mitochondrial light strand promoter anti-sense binding Source: MGI
  • sequence-specific DNA binding Source: MGI
  • serine-type endopeptidase activity Source: UniProtKB-HAMAP
  • single-stranded DNA binding Source: MGI
  • single-stranded RNA binding Source: MGI

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Protease, Serine protease

Keywords - Ligandi

ATP-binding, DNA-binding, Nucleotide-binding

Protein family/group databases

MEROPSiS16.002.

Names & Taxonomyi

Protein namesi
Recommended name:
Lon protease homolog, mitochondrialUniRule annotation (EC:3.4.21.-UniRule annotation)
Alternative name(s):
Lon protease-like proteinUniRule annotation
Short name:
LONPUniRule annotation
Mitochondrial ATP-dependent protease LonUniRule annotation
Serine protease 15UniRule annotation
Gene namesi
Name:Lonp1
Synonyms:Prss15
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 17

Organism-specific databases

MGIiMGI:1921392. Lonp1.

Subcellular locationi

  • Mitochondrion matrix UniRule annotation1 Publication

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Chemistry databases

ChEMBLiCHEMBL3259486.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 65MitochondrionUniRule annotationAdd BLAST65
ChainiPRO_000025496166 – 949Lon protease homolog, mitochondrialAdd BLAST884

Proteomic databases

EPDiQ8CGK3.
MaxQBiQ8CGK3.
PaxDbiQ8CGK3.
PeptideAtlasiQ8CGK3.
PRIDEiQ8CGK3.

2D gel databases

REPRODUCTION-2DPAGEQ8CGK3.
Q9DBP9.

PTM databases

iPTMnetiQ8CGK3.
PhosphoSitePlusiQ8CGK3.
SwissPalmiQ8CGK3.

Expressioni

Tissue specificityi

Detected in liver > heart > kidney > testis.1 Publication

Gene expression databases

BgeeiENSMUSG00000041168.
CleanExiMM_LONP1.
GenevisibleiQ8CGK3. MM.

Interactioni

Subunit structurei

Homohexamer or homoheptamer. Organized in a ring with a central cavity. DNA and RNA binding is stimulated by substrate and inhibited by ATP binding. Interacts with PEO1 and mitochondrial DNA polymerase subunit POLG.UniRule annotation

GO - Molecular functioni

Protein-protein interaction databases

BioGridi216521. 1 interactor.
IntActiQ8CGK3. 3 interactors.
MINTiMINT-1540850.
STRINGi10090.ENSMUSP00000041814.

Structurei

3D structure databases

ProteinModelPortaliQ8CGK3.
SMRiQ8CGK3.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini112 – 357Lon N-terminalPROSITE-ProRule annotationAdd BLAST246
Domaini748 – 938Lon proteolyticPROSITE-ProRule annotationAdd BLAST191

Sequence similaritiesi

Belongs to the peptidase S16 family.UniRule annotation
Contains 1 Lon N-terminal domain.PROSITE-ProRule annotation
Contains 1 Lon proteolytic domain.PROSITE-ProRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2004. Eukaryota.
COG0466. LUCA.
GeneTreeiENSGT00530000063553.
HOGENOMiHOG000261409.
HOVERGENiHBG000798.
InParanoidiQ8CGK3.
KOiK08675.
OMAiVGAYKGE.
OrthoDBiEOG091G01TN.
PhylomeDBiQ8CGK3.
TreeFamiTF105001.

Family and domain databases

Gene3Di3.30.230.10. 1 hit.
3.40.50.300. 1 hit.
HAMAPiMF_03120. lonm_euk. 1 hit.
InterProiIPR003593. AAA+_ATPase.
IPR003959. ATPase_AAA_core.
IPR004815. Lon_bac/euk-typ.
IPR027065. Lon_Prtase.
IPR003111. LON_substr-bd_dom.
IPR027503. Lonm_euk.
IPR027417. P-loop_NTPase.
IPR008269. Pept_S16_C.
IPR008268. Peptidase_S16_AS.
IPR015947. PUA-like_domain.
IPR020568. Ribosomal_S5_D2-typ_fold.
IPR014721. Ribosomal_S5_D2-typ_fold_subgr.
[Graphical view]
PANTHERiPTHR10046. PTHR10046. 2 hits.
PfamiPF00004. AAA. 1 hit.
PF05362. Lon_C. 1 hit.
PF02190. LON_substr_bdg. 1 hit.
[Graphical view]
SMARTiSM00382. AAA. 1 hit.
SM00464. LON. 1 hit.
[Graphical view]
SUPFAMiSSF52540. SSF52540. 1 hit.
SSF54211. SSF54211. 1 hit.
SSF88697. SSF88697. 1 hit.
TIGRFAMsiTIGR00763. lon. 1 hit.
PROSITEiPS51787. LON_N. 1 hit.
PS51786. LON_PROTEOLYTIC. 1 hit.
PS01046. LON_SER. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

Q8CGK3-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAASTGYVRL WAAARCWVLR RPLLAVTGGR VPSASGSWLR RGCRACDMSA
60 70 80 90 100
PWGGRVLPGG VQWRGLWDSG NRGGSDETSE GGAEDGATAS TGEGPVVTAL
110 120 130 140 150
APMTVPDVFP HLPLIAITRN PVFPRFIKIV EVKNKKLVEL LRRKVRLAQP
160 170 180 190 200
YVGVFLKRDD NNESDVVESL DEIYHTGTFA QIHEMQDLGD KLRMIVTGHR
210 220 230 240 250
RIHISRQLEV EPEGLEPEAE KQKSRRKLKR GKKEVEDELG PKPQLEMVTE
260 270 280 290 300
AATDTSKEVL MVEVENVAHE DFQVTEEVKA LTAEIVKTIR DIIALNPLYR
310 320 330 340 350
ESVLQMMQAG QRVVDNPIYL SDMGAALTGA ESHELQDVLE ETNILKRLYK
360 370 380 390 400
ALSLLKKEFE LSKLQQRLGR EVEEKIKQTH RKYLLQEQLK IIKKELGLEK
410 420 430 440 450
DDKDAIEEKF RERLRELVVP KHVMDVVDEE LSKLALLDNH SSEFNVTRNY
460 470 480 490 500
LDWLTSIPWG RQSDENLDLA RAQAVLEEDH YGMEDVKKRV LEFIAVSQLR
510 520 530 540 550
GSTQGKILCF HGPPGVGKTS IARSIARALG REYFRFSVGG MTDVAEIKGH
560 570 580 590 600
RRTYVGAMPG KIIQCLKKTK TENPLVLIDE VDKIGRGYQG DPSSALLELL
610 620 630 640 650
DPEQNANFLD HYLDVPVDLS KVLFICTANV IDTIPEPLRD RMEMINVSGY
660 670 680 690 700
VAQEKLAIAE RYLVPQARTL CGLDESKAQL SAAVLTLLIK QYCRESGVRN
710 720 730 740 750
LQKQVEKVLR KAAYKIVSGE AQTVQVTPEN LQDFVGKPVF TVERMYEVTP
760 770 780 790 800
PGVVMGLAWT AMGGSTLFVE TSLRRPQPSG SKEDKDGSLE VTGQLGDVMK
810 820 830 840 850
ESARIAYTYA RAFLMEQDPE NDFLVTSHIH LHVPEGATPK DGPSAGCTIV
860 870 880 890 900
TALLSLALGQ PVLQNLAMTG EVSLTGKVLP VGGIKEKTIA AKRAGVTCII
910 920 930 940
LPAENRKDYS DLAPFITEGL EVHFVEHYRD IFPIAFPRRE HREALAVER
Length:949
Mass (Da):105,843
Last modified:October 31, 2006 - v2
Checksum:i4622E900C8D90369
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti90S → G in BAB23591 (PubMed:16141072).Curated1
Sequence conflicti99A → T in BAE34094 (PubMed:16141072).Curated1
Sequence conflicti202I → T in BAB23591 (PubMed:16141072).Curated1
Sequence conflicti214G → R in AAN85210 (PubMed:12657466).Curated1
Sequence conflicti240G → S in BAE34094 (PubMed:16141072).Curated1
Sequence conflicti248V → L in BAE34094 (PubMed:16141072).Curated1
Sequence conflicti271D → Y in BAB23591 (PubMed:16141072).Curated1
Sequence conflicti368L → P in BAB23591 (PubMed:16141072).Curated1
Sequence conflicti424M → I in BAE36666 (PubMed:16141072).Curated1
Sequence conflicti449N → K in BAE34972 (PubMed:16141072).Curated1
Sequence conflicti636E → K in BAE34094 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY190302 mRNA. Translation: AAN85210.1.
AK004820 mRNA. Translation: BAB23591.1.
AK157474 mRNA. Translation: BAE34094.1.
AK159302 mRNA. Translation: BAE34972.1.
AK160071 mRNA. Translation: BAE35606.1.
AK161983 mRNA. Translation: BAE36666.1.
CCDSiCCDS28910.1.
RefSeqiNP_083058.2. NM_028782.2.
UniGeneiMm.329136.

Genome annotation databases

EnsembliENSMUST00000047226; ENSMUSP00000041814; ENSMUSG00000041168.
GeneIDi74142.
KEGGimmu:74142.
UCSCiuc008dcp.2. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY190302 mRNA. Translation: AAN85210.1.
AK004820 mRNA. Translation: BAB23591.1.
AK157474 mRNA. Translation: BAE34094.1.
AK159302 mRNA. Translation: BAE34972.1.
AK160071 mRNA. Translation: BAE35606.1.
AK161983 mRNA. Translation: BAE36666.1.
CCDSiCCDS28910.1.
RefSeqiNP_083058.2. NM_028782.2.
UniGeneiMm.329136.

3D structure databases

ProteinModelPortaliQ8CGK3.
SMRiQ8CGK3.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi216521. 1 interactor.
IntActiQ8CGK3. 3 interactors.
MINTiMINT-1540850.
STRINGi10090.ENSMUSP00000041814.

Chemistry databases

ChEMBLiCHEMBL3259486.

Protein family/group databases

MEROPSiS16.002.

PTM databases

iPTMnetiQ8CGK3.
PhosphoSitePlusiQ8CGK3.
SwissPalmiQ8CGK3.

2D gel databases

REPRODUCTION-2DPAGEQ8CGK3.
Q9DBP9.

Proteomic databases

EPDiQ8CGK3.
MaxQBiQ8CGK3.
PaxDbiQ8CGK3.
PeptideAtlasiQ8CGK3.
PRIDEiQ8CGK3.

Protocols and materials databases

DNASUi74142.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000047226; ENSMUSP00000041814; ENSMUSG00000041168.
GeneIDi74142.
KEGGimmu:74142.
UCSCiuc008dcp.2. mouse.

Organism-specific databases

CTDi9361.
MGIiMGI:1921392. Lonp1.

Phylogenomic databases

eggNOGiKOG2004. Eukaryota.
COG0466. LUCA.
GeneTreeiENSGT00530000063553.
HOGENOMiHOG000261409.
HOVERGENiHBG000798.
InParanoidiQ8CGK3.
KOiK08675.
OMAiVGAYKGE.
OrthoDBiEOG091G01TN.
PhylomeDBiQ8CGK3.
TreeFamiTF105001.

Miscellaneous databases

ChiTaRSiLonp1. mouse.
PROiQ8CGK3.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000041168.
CleanExiMM_LONP1.
GenevisibleiQ8CGK3. MM.

Family and domain databases

Gene3Di3.30.230.10. 1 hit.
3.40.50.300. 1 hit.
HAMAPiMF_03120. lonm_euk. 1 hit.
InterProiIPR003593. AAA+_ATPase.
IPR003959. ATPase_AAA_core.
IPR004815. Lon_bac/euk-typ.
IPR027065. Lon_Prtase.
IPR003111. LON_substr-bd_dom.
IPR027503. Lonm_euk.
IPR027417. P-loop_NTPase.
IPR008269. Pept_S16_C.
IPR008268. Peptidase_S16_AS.
IPR015947. PUA-like_domain.
IPR020568. Ribosomal_S5_D2-typ_fold.
IPR014721. Ribosomal_S5_D2-typ_fold_subgr.
[Graphical view]
PANTHERiPTHR10046. PTHR10046. 2 hits.
PfamiPF00004. AAA. 1 hit.
PF05362. Lon_C. 1 hit.
PF02190. LON_substr_bdg. 1 hit.
[Graphical view]
SMARTiSM00382. AAA. 1 hit.
SM00464. LON. 1 hit.
[Graphical view]
SUPFAMiSSF52540. SSF52540. 1 hit.
SSF54211. SSF54211. 1 hit.
SSF88697. SSF88697. 1 hit.
TIGRFAMsiTIGR00763. lon. 1 hit.
PROSITEiPS51787. LON_N. 1 hit.
PS51786. LON_PROTEOLYTIC. 1 hit.
PS01046. LON_SER. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiLONM_MOUSE
AccessioniPrimary (citable) accession number: Q8CGK3
Secondary accession number(s): Q3TSK9
, Q3TVL2, Q3TXE4, Q3TZW3, Q9DBP9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 31, 2006
Last sequence update: October 31, 2006
Last modified: November 2, 2016
This is version 121 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. Peptidase families
    Classification of peptidase families and list of entries
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.