Lonp1

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 annotation2 Publications

Catalytic activityⁱ

Hydrolysis of proteins in presence of ATP.UniRule annotation

Sites

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Active siteⁱ	845	UniRule annotation		1
Active siteⁱ	888	UniRule annotation		1

Regions

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Nucleotide bindingⁱ	513 – 520	ATPUniRule annotation		8

GO - Molecular functionⁱ

ADP binding Source: Ensembl
ATP binding Source: UniProtKB-KW
ATP-dependent peptidase activity
Source: RGD
Inferred from direct assayⁱ
- 12752449
DNA polymerase binding Source: Ensembl
G-quadruplex DNA binding Source: Ensembl
mitochondrial promoter sequence-specific DNA binding Source: Ensembl
sequence-specific DNA binding Source: Ensembl
serine-type endopeptidase activity Source: InterPro
single-stranded DNA binding Source: Ensembl
single-stranded RNA binding Source: Ensembl

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

aging
Source: RGD
Inferred from expression patternⁱ
- 15560797
cellular protein-containing complex assembly
Source: RGD
Inferred from mutant phenotypeⁱ
- 12082077
cellular response to oxidative stress Source: Ensembl
mitochondrion organization
Source: RGD
Inferred from expression patternⁱ
- 10050756
oxidation-dependent protein catabolic process Source: Ensembl
protein homooligomerization Source: Ensembl
protein quality control for misfolded or incompletely synthesized proteins Source: GO_Central
proteolysis
Source: RGD
Inferred from direct assayⁱ
- 12752449
response to aluminum ion
Source: RGD
Inferred from expression patternⁱ
- 19010380
response to hormone
Source: RGD
Inferred from expression patternⁱ
- 10050756
response to hypoxia
Source: RGD
Inferred from expression patternⁱ
- 12082077

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	DNA-binding, Hydrolase, Protease, Serine protease
Ligand	ATP-binding, Nucleotide-binding

Protein family/group databases

MEROPS protease database More...MEROPSⁱ	S16.002

MEROPSⁱ

S16.002

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Lon protease homolog, mitochondrialUniRule annotation (EC:3.4.21.53UniRule 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:Lon, Prss15
Organismⁱ	Rattus norvegicus (Rat)
Taxonomic identifierⁱ	10116 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Rattus
Proteomesⁱ	UP000002494 Componentⁱ: Chromosome 9

Organism-specific databases

Rat genome database More...RGDⁱ	621598 Lonp1

RGDⁱ

621598 Lonp1

Keywords - Cellular componentⁱ

Mitochondrion

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Transit peptideⁱ	1 – 65	MitochondrionUniRule annotationAdd BLAST		65
ChainⁱPRO_0000254962	66 – 950	Lon protease homolog, mitochondrialAdd BLAST		885

Proteomic databases

PaxDbⁱ	Q924S5
PRIDEⁱ	Q924S5

PTM databases

iPTMnetⁱ	Q924S5
PhosphoSitePlusⁱ	Q924S5

Expressionⁱ

Inductionⁱ

By hypoxia or ER stress.1 Publication

Gene expression databases

Bgeeⁱ	ENSRNOG00000046502 Expressed in 10 organ(s), highest expression level in adult mammalian kidney
Genevisibleⁱ	Q924S5 RN

Interactionⁱ

Subunit structureⁱ

Homohexamer. Organized in a ring with a central cavity. The ATP-binding and proteolytic domains (AP-domain) form a hexameric chamber, while the N-terminal domain is arranged as a trimer of dimers. DNA and RNA binding is stimulated by substrate and inhibited by ATP binding. Interacts with TWNK and mitochondrial DNA polymerase subunit POLG.UniRule annotation

GO - Molecular functionⁱ

DNA polymerase binding Source: Ensembl

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

IntActⁱ	Q924S5, 1 interactor
STRINGⁱ	10116.ENSRNOP00000066618

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	Q924S5
SMRⁱ	Q924S5
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Domains and Repeats

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Domainⁱ	112 – 358	Lon N-terminalPROSITE-ProRule annotationAdd BLAST		247
Domainⁱ	749 – 939	Lon proteolyticPROSITE-ProRule annotationAdd BLAST		191

Sequence similaritiesⁱ

Belongs to the peptidase S16 family.UniRule annotation

Keywords - Domainⁱ

Transit peptide

Phylogenomic databases

eggNOGⁱ	KOG2004 Eukaryota COG0466 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00530000063553
HOVERGENⁱ	HBG000798
InParanoidⁱ	Q924S5
KEGG Orthology (KO) More...KOⁱ	K08675
OMAⁱ	RSAENFG
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G01TN
PhylomeDBⁱ	Q924S5

Family and domain databases

Gene3Dⁱ	3.30.230.10, 1 hit
HAMAPⁱ	MF_03120 lonm_euk, 1 hit
InterProⁱ	View 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 IPR027503 Lonm_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
PANTHERⁱ	PTHR43718 PTHR43718, 1 hit
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF00004 AAA, 1 hit PF05362 Lon_C, 1 hit PF02190 LON_substr_bdg, 1 hit
PIRSFⁱ	PIRSF001174 Lon_proteas, 1 hit
SMARTⁱ	View protein in SMART SM00382 AAA, 1 hit SM00464 LON, 1 hit
SUPFAMⁱ	SSF52540 SSF52540, 1 hit SSF54211 SSF54211, 1 hit SSF88697 SSF88697, 1 hit
TIGRFAMsⁱ	TIGR00763 lon, 1 hit
PROSITEⁱ	View protein in PROSITE PS51787 LON_N, 1 hit PS51786 LON_PROTEOLYTIC, 1 hit PS01046 LON_SER, 1 hit

Sequenceⁱ

Sequence statusⁱ: Complete.

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

Q924S5-1 [UniParc]FASTA Add to basket

« Hide

        10         20         30         40         50
MAASTGYVRL WAAARCWVLR RPLLAVTGGR VPSASGSWLR RGCRVCDTST 
        60         70         80         90        100
PWGGRVPMGG GQWRGLWDAG SRGGSDETSE GGVEDGATAS SGEGPVVTAL 
       110        120        130        140        150
APMTVPDVFP HLPLIAISRN PVFPRFIKIV EVKNKKLVEL LRRKVRLAQP 
       160        170        180        190        200
YVGVFLKRDD NNESDVVESL DEIYHTGTFA QIHEMQDLGD KLRMIVTGHR 
       210        220        230        240        250
RIHISRQLEV EPEGLEPEAE NKQKSRRKLK RGKKEVGDEL GAKPQLEMVT 
       260        270        280        290        300
EATSDTSKEV LMVEVENVAH EDFQVTEEVK ALTAEIVKTI RDIIALNPLY 
       310        320        330        340        350
RESVLQMMQA GQRVVDNPIY LSDMGAALTG AESHELQDVL EETNILKRLY 
       360        370        380        390        400
KALSLLKKEF ELSKLQQRLG REVEEKIKQT HRKYLLQEQL KIIKKELGLE 
       410        420        430        440        450
KDDKDAIEEK FRERLKELVV PKHVMDVVDE ELSKLALLDN HSSEFNVTRN 
       460        470        480        490        500
YLDWLTSIPW GRQSDENLDL ARAQSVLEED HYGMEDVKKR VLEFIAVSQL 
       510        520        530        540        550
RGSTQGKILC FHGPPGVGKT SIARSIARAL GREYFRFSVG GMTDVAEIKG 
       560        570        580        590        600
HRRTYVGAMP GKIIQCLKKT KTENPLVLID EVDKIGRGYQ GDPSSALLEL 
       610        620        630        640        650
LDPEQNANFL DHYLDVPVDL SKVLFICTAN VTDTIPEPLR DRMEMINVSG 
       660        670        680        690        700
YVAQEKLAIA ERYLVPQART LCGLDESKAQ LSATVLTLLI KQYCRESGVR 
       710        720        730        740        750
NLQKQVEKVL RKAAYKIVSG EAQTVHVTPE NLQDFVGKPV FTVERMYDVT 
       760        770        780        790        800
PPGVVMGLAW TAMGGSTLFV ETSLRRPQPS GSKEDKDGSL EVTGQLGDVM 
       810        820        830        840        850
KESARIAYTF ARAFLMEQDP ENDFLVTSHI HLHVPEGATP KDGPSAGCTI 
       860        870        880        890        900
VTALLSLALG QPVLQNLAMT GEVSLTGKVL PVGGIKEKTI AAKRAGVTCI 
       910        920        930        940        950
ILPAENRKDF SDLAPFITEG LEVHFVEHYR DIFRIAFPLR EHQEALAVER

Length:950

Mass (Da):105,793

Last modified:December 1, 2001 - v1

Checksum:ⁱD505C3D851B6F0E7

GO

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AB064323 mRNA Translation: BAB62423.1
NCBI Reference Sequences More...RefSeqⁱ	NP_596895.1, NM_133404.1
UniGeneⁱ	Rn.146805

Genome annotation databases

Ensemblⁱ	ENSRNOT00000074253; ENSRNOP00000066618; ENSRNOG00000046502
GeneIDⁱ	170916
KEGGⁱ	rno:170916

Similar proteinsⁱ

90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
Q924S5	Lon protease homolog, mitochondrial		MOUSE		949	UniRef90_Q8CGK3
Lon protease homolog, mitochondrial		NEOLE		955
Lon protease homolog, mitochondrial		CRIGR		842
UPI00042ABBCC		PERMB		954
UPI000B4F1CEB		MERUN		954
+10

Protein	Similar proteins		Species	Score	Length	Source
Q924S5	Lon protease homolog, mitochondrial		MOUSE		949	UniRef50_Q8CGK3
Lon protease homolog, mitochondrial		NEOLE		955
Lon protease homolog, mitochondrial		CRIGR		842
Lon protease homolog, mitochondrial		LARCR		1011
Lon protease homolog, mitochondrial		GASAC		903
+306

Entry informationⁱ

Entry nameⁱ	LONM_RAT
Accessionⁱ	Q924S5Primary (citable) accession number: Q924S5
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	October 31, 2006
	Last sequence update:	December 1, 2001
	Last modified:	September 12, 2018
	This is version 120 of the entry and version 1 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program

Miscellaneousⁱ

Keywords - Technical termⁱ

Complete proteome, Reference proteome

UniProtKB

UniParc

Help

UniRef

Proteomes

Annotation systems

Supporting data

UniProtKB - Q924S5 (LONM_RAT)

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Lon protease homolog, mitochondrial

Select a section on the left to see content.

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Sites

Regions

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

Protein family/group databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Mitochondrion

Cytosol

Mitochondrion

Nucleus

Keywords - Cellular componenti

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

GO - Molecular functioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Keywords - Domaini

Phylogenomic databases

Family and domain databases

Sequence databases

Genome annotation databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

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Functionⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

PTM / Processingⁱ

Expressionⁱ

Interactionⁱ

GO - Molecular functionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Domainⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ