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Protein

Lon protease homolog, mitochondrial

Gene

LONP1

Organism
Homo sapiens (Human)
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. Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein.UniRule annotation4 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei855UniRule annotation1
Active sitei898UniRule annotation1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi523 – 530ATPUniRule annotation8

GO - Molecular functioni

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

GO - Biological processi

  • aging Source: Ensembl
  • cellular response to oxidative stress Source: UniProtKB
  • chaperone-mediated protein complex assembly Source: GO_Central
  • misfolded or incompletely synthesized protein catabolic process Source: GO_Central
  • mitochondrial DNA metabolic process Source: UniProtKB
  • mitochondrial genome maintenance Source: UniProtKB
  • mitochondrion organization Source: UniProtKB
  • oxidation-dependent protein catabolic process Source: UniProtKB
  • protein homooligomerization Source: UniProtKB
  • proteolysis involved in cellular protein catabolic process Source: UniProtKB
  • regulation of mitochondrial DNA replication Source: UniProtKB-HAMAP
  • response to aluminum ion Source: Ensembl
  • response to hormone Source: Ensembl
  • response to hypoxia Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Protease, Serine protease

Keywords - Ligandi

ATP-binding, DNA-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciZFISH:HS06887-MONOMER.
BRENDAi3.4.21.53. 2681.

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):
LONHs
Lon protease-like proteinUniRule annotation
Short name:
LONPUniRule annotation
Mitochondrial ATP-dependent protease LonUniRule annotation
Serine protease 15UniRule annotation
Gene namesi
Name:LONP1UniRule annotation
Synonyms:PRSS15
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

HGNCiHGNC:9479. LONP1.

Subcellular locationi

  • Mitochondrion matrix UniRule annotation1 Publication

GO - Cellular componenti

  • cytoplasm Source: HPA
  • membrane Source: UniProtKB
  • mitochondrial matrix Source: UniProtKB
  • mitochondrial nucleoid Source: BHF-UCL
  • mitochondrion Source: UniProtKB
  • nucleoplasm Source: HPA
  • nucleus Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

CODAS syndrome (CODASS)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA rare syndrome characterized by the combination of cerebral, ocular, dental, auricular, and skeletal features. These include developmental delay, craniofacial anomalies, cataracts, ptosis, median nasal groove, delayed tooth eruption, hearing loss, short stature, delayed epiphyseal ossification, metaphyseal hip dysplasia, and vertebral coronal clefts.
See also OMIM:600373
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_073338476E → A in CODASS. 1 Publication1
Natural variantiVAR_073339631S → Y in CODASS. 1 Publication1
Natural variantiVAR_073340670A → V in CODASS. 1 PublicationCorresponds to variant rs770036526dbSNPEnsembl.1
Natural variantiVAR_073341672R → C in CODASS. 1 PublicationCorresponds to variant rs777009012dbSNPEnsembl.1
Natural variantiVAR_073342676P → S in CODASS. 1 Publication1
Natural variantiVAR_073343679R → H in CODASS. 1 PublicationCorresponds to variant rs549574673dbSNPEnsembl.1
Natural variantiVAR_073344721R → G in CODASS. 1 PublicationCorresponds to variant rs147588238dbSNPEnsembl.1
Natural variantiVAR_073345724A → V in CODASS. 1 Publication1
Natural variantiVAR_073346749P → S in CODASS. 1 Publication1
Natural variantiVAR_073347767G → E in CODASS. 1 PublicationCorresponds to variant rs562553348dbSNPEnsembl.1
Natural variantiVAR_073348927Missing in CODASS. 1 Publication1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi855S → A: Lacks both ATPase and protease activity, but retains DNA binding activity. 1 Publication1

Keywords - Diseasei

Cataract, Deafness, Disease mutation, Dwarfism

Organism-specific databases

DisGeNETi9361.
MalaCardsiLONP1.
MIMi600373. phenotype.
OpenTargetsiENSG00000196365.
PharmGKBiPA162394145.

Polymorphism and mutation databases

DMDMi12644239.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 67MitochondrionUniRule annotationCombined sourcesAdd BLAST67
ChainiPRO_000002673468 – 959Lon protease homolog, mitochondrialAdd BLAST892

Proteomic databases

EPDiP36776.
MaxQBiP36776.
PaxDbiP36776.
PeptideAtlasiP36776.
PRIDEiP36776.

PTM databases

iPTMnetiP36776.
PhosphoSitePlusiP36776.
SwissPalmiP36776.

Expressioni

Tissue specificityi

Duodenum, heart, lung and liver, but not thymus.

Gene expression databases

BgeeiENSG00000196365.
CleanExiHS_LONP1.
ExpressionAtlasiP36776. baseline and differential.
GenevisibleiP36776. HS.

Organism-specific databases

HPAiHPA002034.
HPA002192.

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 annotation2 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
CSN2P026666EBI-357448,EBI-5260183From a different organism.

GO - Molecular functioni

  • DNA polymerase binding Source: UniProtKB

Protein-protein interaction databases

BioGridi114762. 59 interactors.
IntActiP36776. 36 interactors.
MINTiMINT-3014242.
STRINGi9606.ENSP00000353826.

Structurei

Secondary structure

1959
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi764 – 786Combined sources23
Beta strandi799 – 804Combined sources6
Helixi808 – 828Combined sources21
Helixi834 – 837Combined sources4
Beta strandi839 – 843Combined sources5
Turni850 – 852Combined sources3
Helixi853 – 856Combined sources4
Helixi857 – 869Combined sources13
Beta strandi877 – 879Combined sources3
Beta strandi887 – 890Combined sources4
Helixi895 – 904Combined sources10
Beta strandi909 – 913Combined sources5
Helixi914 – 916Combined sources3
Helixi917 – 921Combined sources5
Helixi925 – 928Combined sources4
Beta strandi932 – 938Combined sources7
Helixi939 – 946Combined sources8

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2X36X-ray2.00A/B/C/D/E/F753-959[»]
ProteinModelPortaliP36776.
SMRiP36776.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP36776.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini124 – 368Lon N-terminalPROSITE-ProRule annotationAdd BLAST245
Domaini759 – 949Lon 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.
InParanoidiP36776.
KOiK08675.
OMAiVGAYKGE.
OrthoDBiEOG091G01TN.
PhylomeDBiP36776.
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]

Sequences (3)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P36776-1) [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
MAASTGYVRL WGAARCWVLR RPMLAAAGGR VPTAAGAWLL RGQRTCDASP
60 70 80 90 100
PWALWGRGPA IGGQWRGFWE ASSRGGGAFS GGEDASEGGA EEGAGGAGGS
110 120 130 140 150
AGAGEGPVIT ALTPMTIPDV FPHLPLIAIT RNPVFPRFIK IIEVKNKKLV
160 170 180 190 200
ELLRRKVRLA QPYVGVFLKR DDSNESDVVE SLDEIYHTGT FAQIHEMQDL
210 220 230 240 250
GDKLRMIVMG HRRVHISRQL EVEPEEPEAE NKHKPRRKSK RGKKEAEDEL
260 270 280 290 300
SARHPAELAM EPTPELPAEV LMVEVENVVH EDFQVTEEVK ALTAEIVKTI
310 320 330 340 350
RDIIALNPLY RESVLQMMQA GQRVVDNPIY LSDMGAALTG AESHELQDVL
360 370 380 390 400
EETNIPKRLY KALSLLKKEF ELSKLQQRLG REVEEKIKQT HRKYLLQEQL
410 420 430 440 450
KIIKKELGLE KDDKDAIEEK FRERLKELVV PKHVMDVVDE ELSKLGLLDN
460 470 480 490 500
HSSEFNVTRN YLDWLTSIPW GKYSNENLDL ARAQAVLEED HYGMEDVKKR
510 520 530 540 550
ILEFIAVSQL RGSTQGKILC FYGPPGVGKT SIARSIARAL NREYFRFSVG
560 570 580 590 600
GMTDVAEIKG HRRTYVGAMP GKIIQCLKKT KTENPLILID EVDKIGRGYQ
610 620 630 640 650
GDPSSALLEL LDPEQNANFL DHYLDVPVDL SKVLFICTAN VTDTIPEPLR
660 670 680 690 700
DRMEMINVSG YVAQEKLAIA ERYLVPQARA LCGLDESKAK LSSDVLTLLI
710 720 730 740 750
KQYCRESGVR NLQKQVEKVL RKSAYKIVSG EAESVEVTPE NLQDFVGKPV
760 770 780 790 800
FTVERMYDVT PPGVVMGLAW TAMGGSTLFV ETSLRRPQDK DAKGDKDGSL
810 820 830 840 850
EVTGQLGEVM KESARIAYTF ARAFLMQHAP ANDYLVTSHI HLHVPEGATP
860 870 880 890 900
KDGPSAGCTI VTALLSLAMG RPVRQNLAMT GEVSLTGKIL PVGGIKEKTI
910 920 930 940 950
AAKRAGVTCI VLPAENKKDF YDLAAFITEG LEVHFVEHYR EIFDIAFPDE

QAEALAVER
Length:959
Mass (Da):106,489
Last modified:December 1, 2000 - v2
Checksum:iB5E03D9C27C220FF
GO
Isoform 2 (identifier: P36776-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     42-105: Missing.

Note: No experimental confirmation available.
Show »
Length:895
Mass (Da):100,397
Checksum:iEF213C4E36C69AE1
GO
Isoform 3 (identifier: P36776-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-196: Missing.

Note: No experimental confirmation available.
Show »
Length:763
Mass (Da):85,642
Checksum:i3FEBB8DEEA651D7A
GO

Sequence cautioni

The sequence CAA52291 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti1 – 55MAAST…PWALW → MAGLWRRALATCDCGERRGA GCCGGRCWPRRGAGSHCSRS VVAPRPADLRRLSSLGTV in AAA61616 (PubMed:8248235).CuratedAdd BLAST55
Sequence conflicti65 – 66WR → CG in AAA61616 (PubMed:8248235).Curated2
Sequence conflicti257 – 258EL → DV in AAA61616 (PubMed:8248235).Curated2
Sequence conflicti423E → G in BAG51690 (PubMed:14702039).Curated1
Sequence conflicti456N → D in AAA61616 (PubMed:8248235).Curated1
Sequence conflicti501I → V in BAG51690 (PubMed:14702039).Curated1
Sequence conflicti556A → T in AAA61616 (PubMed:8248235).Curated1
Sequence conflicti842L → P in AAA61616 (PubMed:8248235).Curated1
Sequence conflicti859T → A in AAA61616 (PubMed:8248235).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05156487E → D.Corresponds to variant rs34413649dbSNPEnsembl.1
Natural variantiVAR_051565241R → Q.Corresponds to variant rs11085147dbSNPEnsembl.1
Natural variantiVAR_073338476E → A in CODASS. 1 Publication1
Natural variantiVAR_073339631S → Y in CODASS. 1 Publication1
Natural variantiVAR_073340670A → V in CODASS. 1 PublicationCorresponds to variant rs770036526dbSNPEnsembl.1
Natural variantiVAR_073341672R → C in CODASS. 1 PublicationCorresponds to variant rs777009012dbSNPEnsembl.1
Natural variantiVAR_073342676P → S in CODASS. 1 Publication1
Natural variantiVAR_073343679R → H in CODASS. 1 PublicationCorresponds to variant rs549574673dbSNPEnsembl.1
Natural variantiVAR_073344721R → G in CODASS. 1 PublicationCorresponds to variant rs147588238dbSNPEnsembl.1
Natural variantiVAR_073345724A → V in CODASS. 1 Publication1
Natural variantiVAR_073346749P → S in CODASS. 1 Publication1
Natural variantiVAR_073347767G → E in CODASS. 1 PublicationCorresponds to variant rs562553348dbSNPEnsembl.1
Natural variantiVAR_067708829A → T.Corresponds to variant rs35804229dbSNPEnsembl.1
Natural variantiVAR_067709911V → I.2 PublicationsCorresponds to variant rs1062373dbSNPEnsembl.1
Natural variantiVAR_073348927Missing in CODASS. 1 Publication1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0553101 – 196Missing in isoform 3. 1 PublicationAdd BLAST196
Alternative sequenceiVSP_05461742 – 105Missing in isoform 2. 1 PublicationAdd BLAST64

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U02389 mRNA. Translation: AAA61616.1.
HQ204946 Genomic DNA. Translation: ADP90374.1.
HQ204947 Genomic DNA. Translation: ADP90375.1.
HQ204948 Genomic DNA. Translation: ADP90376.1.
HQ204949 Genomic DNA. Translation: ADP90377.1.
HQ204950 Genomic DNA. Translation: ADP90378.1.
HQ204951 Genomic DNA. Translation: ADP90379.1.
HQ204952 Genomic DNA. Translation: ADP90380.1.
HQ204953 Genomic DNA. Translation: ADP90381.1.
HQ204954 Genomic DNA. Translation: ADP90382.1.
HQ204955 Genomic DNA. Translation: ADP90383.1.
HQ204956 Genomic DNA. Translation: ADP90384.1.
HQ204957 Genomic DNA. Translation: ADP90385.1.
HQ204958 Genomic DNA. Translation: ADP90386.1.
HQ204959 Genomic DNA. Translation: ADP90387.1.
HQ204960 Genomic DNA. Translation: ADP90388.1.
HQ204961 Genomic DNA. Translation: ADP90389.1.
HQ204962 Genomic DNA. Translation: ADP90390.1.
HQ204963 Genomic DNA. Translation: ADP90391.1.
HQ204964 Genomic DNA. Translation: ADP90392.1.
HQ204965 Genomic DNA. Translation: ADP90393.1.
HQ204966 Genomic DNA. Translation: ADP90394.1.
HQ204968 Genomic DNA. Translation: ADP90396.1.
HQ204969 Genomic DNA. Translation: ADP90397.1.
HQ204970 Genomic DNA. Translation: ADP90398.1.
HQ204971 Genomic DNA. Translation: ADP90399.1.
HQ204972 Genomic DNA. Translation: ADP90400.1.
HQ204973 Genomic DNA. Translation: ADP90401.1.
HQ204974 Genomic DNA. Translation: ADP90402.1.
HQ204975 Genomic DNA. Translation: ADP90403.1.
HQ204976 Genomic DNA. Translation: ADP90404.1.
HQ204977 Genomic DNA. Translation: ADP90405.1.
HQ204978 Genomic DNA. Translation: ADP90406.1.
HQ204979 Genomic DNA. Translation: ADP90407.1.
HQ204980 Genomic DNA. Translation: ADP90408.1.
HQ204981 Genomic DNA. Translation: ADP90409.1.
HQ204982 Genomic DNA. Translation: ADP90410.1.
HQ204983 Genomic DNA. Translation: ADP90411.1.
HQ204984 Genomic DNA. Translation: ADP90412.1.
HQ204985 Genomic DNA. Translation: ADP90413.1.
AF059309
, AF059296, AF059297, AF059298, AF059299, AF059300, AF059301, AF059302, AF059303, AF059304, AF059305, AF059306, AF059307, AF059308 Genomic DNA. Translation: AAD24414.1.
AK056366 mRNA. Translation: BAG51690.1.
AK096626 mRNA. Translation: BAC04829.1.
AC011499 Genomic DNA. No translation available.
CH471139 Genomic DNA. Translation: EAW69151.1.
CH471139 Genomic DNA. Translation: EAW69154.1.
BC000235 mRNA. Translation: AAH00235.1.
X74215 mRNA. Translation: CAA52291.1. Different initiation.
X76040 mRNA. Translation: CAA53625.1.
CCDSiCCDS12148.1. [P36776-1]
CCDS62507.1. [P36776-3]
CCDS62508.1. [P36776-2]
PIRiS42366.
S57342.
RefSeqiNP_001263408.1. NM_001276479.1. [P36776-2]
NP_001263409.1. NM_001276480.1. [P36776-3]
NP_004784.2. NM_004793.3. [P36776-1]
UniGeneiHs.350265.

Genome annotation databases

EnsembliENST00000360614; ENSP00000353826; ENSG00000196365. [P36776-1]
ENST00000540670; ENSP00000441523; ENSG00000196365. [P36776-3]
ENST00000593119; ENSP00000468541; ENSG00000196365. [P36776-2]
GeneIDi9361.
KEGGihsa:9361.
UCSCiuc002mcx.5. human. [P36776-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U02389 mRNA. Translation: AAA61616.1.
HQ204946 Genomic DNA. Translation: ADP90374.1.
HQ204947 Genomic DNA. Translation: ADP90375.1.
HQ204948 Genomic DNA. Translation: ADP90376.1.
HQ204949 Genomic DNA. Translation: ADP90377.1.
HQ204950 Genomic DNA. Translation: ADP90378.1.
HQ204951 Genomic DNA. Translation: ADP90379.1.
HQ204952 Genomic DNA. Translation: ADP90380.1.
HQ204953 Genomic DNA. Translation: ADP90381.1.
HQ204954 Genomic DNA. Translation: ADP90382.1.
HQ204955 Genomic DNA. Translation: ADP90383.1.
HQ204956 Genomic DNA. Translation: ADP90384.1.
HQ204957 Genomic DNA. Translation: ADP90385.1.
HQ204958 Genomic DNA. Translation: ADP90386.1.
HQ204959 Genomic DNA. Translation: ADP90387.1.
HQ204960 Genomic DNA. Translation: ADP90388.1.
HQ204961 Genomic DNA. Translation: ADP90389.1.
HQ204962 Genomic DNA. Translation: ADP90390.1.
HQ204963 Genomic DNA. Translation: ADP90391.1.
HQ204964 Genomic DNA. Translation: ADP90392.1.
HQ204965 Genomic DNA. Translation: ADP90393.1.
HQ204966 Genomic DNA. Translation: ADP90394.1.
HQ204968 Genomic DNA. Translation: ADP90396.1.
HQ204969 Genomic DNA. Translation: ADP90397.1.
HQ204970 Genomic DNA. Translation: ADP90398.1.
HQ204971 Genomic DNA. Translation: ADP90399.1.
HQ204972 Genomic DNA. Translation: ADP90400.1.
HQ204973 Genomic DNA. Translation: ADP90401.1.
HQ204974 Genomic DNA. Translation: ADP90402.1.
HQ204975 Genomic DNA. Translation: ADP90403.1.
HQ204976 Genomic DNA. Translation: ADP90404.1.
HQ204977 Genomic DNA. Translation: ADP90405.1.
HQ204978 Genomic DNA. Translation: ADP90406.1.
HQ204979 Genomic DNA. Translation: ADP90407.1.
HQ204980 Genomic DNA. Translation: ADP90408.1.
HQ204981 Genomic DNA. Translation: ADP90409.1.
HQ204982 Genomic DNA. Translation: ADP90410.1.
HQ204983 Genomic DNA. Translation: ADP90411.1.
HQ204984 Genomic DNA. Translation: ADP90412.1.
HQ204985 Genomic DNA. Translation: ADP90413.1.
AF059309
, AF059296, AF059297, AF059298, AF059299, AF059300, AF059301, AF059302, AF059303, AF059304, AF059305, AF059306, AF059307, AF059308 Genomic DNA. Translation: AAD24414.1.
AK056366 mRNA. Translation: BAG51690.1.
AK096626 mRNA. Translation: BAC04829.1.
AC011499 Genomic DNA. No translation available.
CH471139 Genomic DNA. Translation: EAW69151.1.
CH471139 Genomic DNA. Translation: EAW69154.1.
BC000235 mRNA. Translation: AAH00235.1.
X74215 mRNA. Translation: CAA52291.1. Different initiation.
X76040 mRNA. Translation: CAA53625.1.
CCDSiCCDS12148.1. [P36776-1]
CCDS62507.1. [P36776-3]
CCDS62508.1. [P36776-2]
PIRiS42366.
S57342.
RefSeqiNP_001263408.1. NM_001276479.1. [P36776-2]
NP_001263409.1. NM_001276480.1. [P36776-3]
NP_004784.2. NM_004793.3. [P36776-1]
UniGeneiHs.350265.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2X36X-ray2.00A/B/C/D/E/F753-959[»]
ProteinModelPortaliP36776.
SMRiP36776.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi114762. 59 interactors.
IntActiP36776. 36 interactors.
MINTiMINT-3014242.
STRINGi9606.ENSP00000353826.

Protein family/group databases

MEROPSiS16.002.

PTM databases

iPTMnetiP36776.
PhosphoSitePlusiP36776.
SwissPalmiP36776.

Polymorphism and mutation databases

DMDMi12644239.

Proteomic databases

EPDiP36776.
MaxQBiP36776.
PaxDbiP36776.
PeptideAtlasiP36776.
PRIDEiP36776.

Protocols and materials databases

DNASUi9361.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000360614; ENSP00000353826; ENSG00000196365. [P36776-1]
ENST00000540670; ENSP00000441523; ENSG00000196365. [P36776-3]
ENST00000593119; ENSP00000468541; ENSG00000196365. [P36776-2]
GeneIDi9361.
KEGGihsa:9361.
UCSCiuc002mcx.5. human. [P36776-1]

Organism-specific databases

CTDi9361.
DisGeNETi9361.
GeneCardsiLONP1.
HGNCiHGNC:9479. LONP1.
HPAiHPA002034.
HPA002192.
MalaCardsiLONP1.
MIMi600373. phenotype.
605490. gene.
neXtProtiNX_P36776.
OpenTargetsiENSG00000196365.
PharmGKBiPA162394145.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2004. Eukaryota.
COG0466. LUCA.
GeneTreeiENSGT00530000063553.
HOGENOMiHOG000261409.
HOVERGENiHBG000798.
InParanoidiP36776.
KOiK08675.
OMAiVGAYKGE.
OrthoDBiEOG091G01TN.
PhylomeDBiP36776.
TreeFamiTF105001.

Enzyme and pathway databases

BioCyciZFISH:HS06887-MONOMER.
BRENDAi3.4.21.53. 2681.

Miscellaneous databases

ChiTaRSiLONP1. human.
EvolutionaryTraceiP36776.
GeneWikiiLONP1.
GenomeRNAii9361.
PROiP36776.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000196365.
CleanExiHS_LONP1.
ExpressionAtlasiP36776. baseline and differential.
GenevisibleiP36776. HS.

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_HUMAN
AccessioniPrimary (citable) accession number: P36776
Secondary accession number(s): B3KPH8
, D6W635, E5KMH8, F5GZ27, P36777, Q8N8K8, Q9UQ95
Entry historyi
Integrated into UniProtKB/Swiss-Prot: June 1, 1994
Last sequence update: December 1, 2000
Last modified: November 30, 2016
This is version 168 of the entry and version 2 of the sequence. [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

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. Peptidase families
    Classification of peptidase families and list of entries
  7. 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.