UniProtKB - P36776 (LONM_HUMAN)
Lon protease homolog, mitochondrial
LONP1
Functioni
Catalytic activityi
- Hydrolysis of proteins in presence of ATP.UniRule annotation EC:3.4.21.53
Activity regulationi
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Active sitei | 855 | UniRule annotation | 1 | |
Active sitei | 898 | UniRule annotation | 1 |
Regions
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Nucleotide bindingi | 523 – 530 | ATPUniRule annotation | 8 |
GO - Molecular functioni
- ADP binding Source: UniProtKB
- ATPase activity Source: UniProtKB
- ATP binding Source: UniProtKB
- ATP-dependent peptidase activity Source: UniProtKB
- DNA polymerase binding Source: UniProtKB
- G-quadruplex DNA binding Source: UniProtKB
- identical protein binding Source: IntAct
- mitochondrial promoter sequence-specific DNA binding Source: UniProtKB
- sequence-specific DNA binding Source: UniProtKB
- serine-type endopeptidase activity Source: UniProtKB-UniRule
- 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
- mitochondrial DNA metabolic process Source: UniProtKB
- mitochondrial genome maintenance Source: UniProtKB
- mitochondrion organization Source: UniProtKB
- oxidation-dependent protein catabolic process Source: UniProtKB
- protein quality control for misfolded or incompletely synthesized proteins Source: GO_Central
- proteolysis involved in cellular protein catabolic process Source: UniProtKB
- response to aluminum ion Source: Ensembl
- response to hormone Source: Ensembl
- response to hypoxia Source: UniProtKB
Keywordsi
Molecular function | DNA-binding, Hydrolase, Protease, Serine protease |
Ligand | ATP-binding, Nucleotide-binding |
Enzyme and pathway databases
BRENDAi | 3.4.21.53, 2681 |
PathwayCommonsi | P36776 |
Protein family/group databases
MEROPSi | S16.002 |
MoonDBi | P36776, Curated |
Names & Taxonomyi
Protein namesi | Recommended name: Lon protease homolog, mitochondrialUniRule annotation (EC:3.4.21.53UniRule 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 | |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:9479, LONP1 |
MIMi | 605490, gene |
neXtProti | NX_P36776 |
VEuPathDBi | HostDB:ENSG00000196365.11 |
Subcellular locationi
Mitochondrion
- Mitochondrion matrix UniRule annotation1 Publication
Cytosol
- cytosol Source: HPA
Mitochondrion
- mitochondrial matrix Source: UniProtKB
- mitochondrial nucleoid Source: BHF-UCL
- mitochondrion Source: UniProtKB
Nucleus
- nucleoplasm Source: HPA
Other locations
- membrane Source: UniProtKB
Keywords - Cellular componenti
MitochondrionPathology & Biotechi
Involvement in diseasei
CODAS syndrome (CODASS)2 Publications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_073338 | 476 | E → A in CODASS. 1 Publication | 1 | |
Natural variantiVAR_073339 | 631 | S → Y in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255248EnsemblClinVar. | 1 | |
Natural variantiVAR_073340 | 670 | A → V in CODASS. 1 PublicationCorresponds to variant dbSNP:rs770036526Ensembl. | 1 | |
Natural variantiVAR_073341 | 672 | R → C in CODASS. 1 PublicationCorresponds to variant dbSNP:rs777009012Ensembl. | 1 | |
Natural variantiVAR_073342 | 676 | P → S in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255247EnsemblClinVar. | 1 | |
Natural variantiVAR_073343 | 679 | R → H in CODASS. 1 PublicationCorresponds to variant dbSNP:rs549574673EnsemblClinVar. | 1 | |
Natural variantiVAR_073344 | 721 | R → G in CODASS. 1 PublicationCorresponds to variant dbSNP:rs147588238Ensembl. | 1 | |
Natural variantiVAR_073345 | 724 | A → V in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255249EnsemblClinVar. | 1 | |
Natural variantiVAR_073346 | 749 | P → S in CODASS. 1 Publication | 1 | |
Natural variantiVAR_073347 | 767 | G → E in CODASS. 1 PublicationCorresponds to variant dbSNP:rs562553348Ensembl. | 1 | |
Natural variantiVAR_073348 | 927 | Missing in CODASS. 1 Publication | 1 |
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 529 | K → R: Abolishes ATPase activity, and presumably ATP-driven protein unfolding, but does not block access to the proteolytic active site or prevent a substrate from binding to it. 1 Publication | 1 | |
Mutagenesisi | 770 | W → A: Has low basal, but normal stimulated ATPase activity, and retains peptidase activity. 1 Publication | 1 | |
Mutagenesisi | 770 | W → P: Has normal basal, but low stimulated ATPase activity, and abolishes peptidase activity. 1 Publication | 1 | |
Mutagenesisi | 855 | S → A: Lacks both ATPase and protease activity, but retains DNA binding activity. 2 Publications | 1 | |
Mutagenesisi | 880 | T → V: Enhances the basal, but not the stimulated ATPase activity. 1 Publication | 1 | |
Mutagenesisi | 893 | G → A: Has low basal, but normal stimulated ATPase activity, and retains peptidase activity. 1 Publication | 1 | |
Mutagenesisi | 893 | G → P: Has normal basal, but low stimulated ATPase activity, and abolishes peptidase activity. 1 Publication | 1 | |
Mutagenesisi | 894 | G → A or S: Enhances the basal, but not the stimulated ATPase activity, and retains peptidase activity. 1 Publication | 1 | |
Mutagenesisi | 894 | G → P: Enhances the basal, but not the stimulated ATPase activity, and abolishes peptidase activity. 1 Publication | 1 |
Keywords - Diseasei
Cataract, Deafness, Disease variant, DwarfismOrganism-specific databases
DisGeNETi | 9361 |
MalaCardsi | LONP1 |
MIMi | 600373, phenotype |
OpenTargetsi | ENSG00000196365 |
Orphaneti | 1458, CODAS syndrome 79243, Pyruvate dehydrogenase E1-alpha deficiency |
PharmGKBi | PA162394145 |
Miscellaneous databases
Pharosi | P36776, Tbio |
Genetic variation databases
BioMutai | LONP1 |
DMDMi | 12644239 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 67 | MitochondrionUniRule annotationCombined sourcesAdd BLAST | 67 | |
ChainiPRO_0000026734 | 68 – 959 | Lon protease homolog, mitochondrialAdd BLAST | 892 |
Proteomic databases
EPDi | P36776 |
jPOSTi | P36776 |
MassIVEi | P36776 |
MaxQBi | P36776 |
PaxDbi | P36776 |
PeptideAtlasi | P36776 |
PRIDEi | P36776 |
ProteomicsDBi | 24918 55221 [P36776-1] |
PTM databases
iPTMneti | P36776 |
MetOSitei | P36776 |
PhosphoSitePlusi | P36776 |
SwissPalmi | P36776 |
Expressioni
Tissue specificityi
Gene expression databases
Bgeei | ENSG00000196365, Expressed in right adrenal gland and 233 other tissues |
ExpressionAtlasi | P36776, baseline and differential |
Genevisiblei | P36776, HS |
Organism-specific databases
HPAi | ENSG00000196365, Tissue enhanced (adrenal) |
Interactioni
Subunit structurei
Homohexamer (PubMed:14739292, PubMed:25369343, PubMed:20222013). Organized in a ring with a central cavity (PubMed:25369343, PubMed:20222013). The ATP-binding and proteolytic domains (AP-domain) form a hexameric chamber, while the N-terminal domain is arranged as a trimer of dimers (PubMed:27632940). DNA and RNA binding is stimulated by substrate and inhibited by ATP binding.
Interacts with TWNK and mitochondrial DNA polymerase subunit POLG (PubMed:14739292).
UniRule annotation4 PublicationsBinary interactionsi
Hide detailsP36776
With | #Exp. | IntAct |
---|---|---|
CSN2 [P02666] from Bos taurus. | 6 | EBI-357448,EBI-5260183 |
LONP1 - isoform 1 [P36776-1]
With | #Exp. | IntAct |
---|---|---|
itself | 3 | EBI-25473602,EBI-25473602 |
GO - Molecular functioni
- DNA polymerase binding Source: UniProtKB
- identical protein binding Source: IntAct
Protein-protein interaction databases
BioGRIDi | 114762, 291 interactors |
IntActi | P36776, 75 interactors |
MINTi | P36776 |
STRINGi | 9606.ENSP00000353826 |
Miscellaneous databases
RNActi | P36776, protein |
Structurei
Secondary structure
3D structure databases
SMRi | P36776 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | P36776 |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 124 – 368 | Lon N-terminalPROSITE-ProRule annotationAdd BLAST | 245 | |
Domaini | 759 – 949 | Lon proteolyticPROSITE-ProRule annotationAdd BLAST | 191 |
Domaini
Sequence similaritiesi
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG2004, Eukaryota |
GeneTreei | ENSGT00530000063553 |
HOGENOMi | CLU_004109_1_1_1 |
InParanoidi | P36776 |
OMAi | ENVAHED |
OrthoDBi | 528132at2759 |
PhylomeDBi | P36776 |
TreeFami | TF105001 |
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 |
Pfami | View protein in Pfam PF00004, AAA, 1 hit PF05362, Lon_C, 1 hit PF02190, LON_substr_bdg, 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 |
s (3+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 3 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 3 described isoforms and 7 potential isoforms that are computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
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
Computationally mapped potential isoform sequencesi
There are 7 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketK7EKE6 | K7EKE6_HUMAN | Lon protease homolog, mitochondrial | LONP1 | 845 | Annotation score: | ||
K7EJE8 | K7EJE8_HUMAN | Lon protease homolog, mitochondrial | LONP1 | 829 | Annotation score: | ||
K7ERR6 | K7ERR6_HUMAN | Lon protease homolog, mitochondrial | LONP1 | 264 | Annotation score: | ||
K7ER27 | K7ER27_HUMAN | Lon protease homolog, mitochondrial | LONP1 | 242 | Annotation score: | ||
K7EQF8 | K7EQF8_HUMAN | Lon protease homolog, mitochondrial | LONP1 | 185 | Annotation score: | ||
K7ER56 | K7ER56_HUMAN | Lon protease homolog, mitochondrial | LONP1 | 210 | Annotation score: | ||
K7ERS1 | K7ERS1_HUMAN | Lon protease homolog, mitochondrial | LONP1 | 86 | Annotation score: |
Sequence cautioni
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 1 – 55 | MAAST…PWALW → MAGLWRRALATCDCGERRGA GCCGGRCWPRRGAGSHCSRS VVAPRPADLRRLSSLGTV in AAA61616 (PubMed:8248235).CuratedAdd BLAST | 55 | |
Sequence conflicti | 65 – 66 | WR → CG in AAA61616 (PubMed:8248235).Curated | 2 | |
Sequence conflicti | 257 – 258 | EL → DV in AAA61616 (PubMed:8248235).Curated | 2 | |
Sequence conflicti | 423 | E → G in BAG51690 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 456 | N → D in AAA61616 (PubMed:8248235).Curated | 1 | |
Sequence conflicti | 501 | I → V in BAG51690 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 556 | A → T in AAA61616 (PubMed:8248235).Curated | 1 | |
Sequence conflicti | 842 | L → P in AAA61616 (PubMed:8248235).Curated | 1 | |
Sequence conflicti | 859 | T → A in AAA61616 (PubMed:8248235).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_051564 | 87 | E → D. Corresponds to variant dbSNP:rs34413649EnsemblClinVar. | 1 | |
Natural variantiVAR_051565 | 241 | R → Q. Corresponds to variant dbSNP:rs11085147EnsemblClinVar. | 1 | |
Natural variantiVAR_073338 | 476 | E → A in CODASS. 1 Publication | 1 | |
Natural variantiVAR_073339 | 631 | S → Y in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255248EnsemblClinVar. | 1 | |
Natural variantiVAR_073340 | 670 | A → V in CODASS. 1 PublicationCorresponds to variant dbSNP:rs770036526Ensembl. | 1 | |
Natural variantiVAR_073341 | 672 | R → C in CODASS. 1 PublicationCorresponds to variant dbSNP:rs777009012Ensembl. | 1 | |
Natural variantiVAR_073342 | 676 | P → S in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255247EnsemblClinVar. | 1 | |
Natural variantiVAR_073343 | 679 | R → H in CODASS. 1 PublicationCorresponds to variant dbSNP:rs549574673EnsemblClinVar. | 1 | |
Natural variantiVAR_073344 | 721 | R → G in CODASS. 1 PublicationCorresponds to variant dbSNP:rs147588238Ensembl. | 1 | |
Natural variantiVAR_073345 | 724 | A → V in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255249EnsemblClinVar. | 1 | |
Natural variantiVAR_073346 | 749 | P → S in CODASS. 1 Publication | 1 | |
Natural variantiVAR_073347 | 767 | G → E in CODASS. 1 PublicationCorresponds to variant dbSNP:rs562553348Ensembl. | 1 | |
Natural variantiVAR_067708 | 829 | A → T. Corresponds to variant dbSNP:rs35804229Ensembl. | 1 | |
Natural variantiVAR_067709 | 911 | V → I2 PublicationsCorresponds to variant dbSNP:rs1062373Ensembl. | 1 | |
Natural variantiVAR_073348 | 927 | Missing in CODASS. 1 Publication | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_055310 | 1 – 196 | Missing in isoform 3. 1 PublicationAdd BLAST | 196 | |
Alternative sequenceiVSP_054617 | 42 – 105 | Missing in isoform 2. 1 PublicationAdd BLAST | 64 |
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 |
CCDSi | CCDS12148.1 [P36776-1] CCDS62507.1 [P36776-3] CCDS62508.1 [P36776-2] |
PIRi | S42366 S57342 |
RefSeqi | NP_001263408.1, NM_001276479.1 [P36776-2] NP_001263409.1, NM_001276480.1 [P36776-3] NP_004784.2, NM_004793.3 [P36776-1] |
Genome annotation databases
Ensembli | ENST00000360614; ENSP00000353826; ENSG00000196365 [P36776-1] ENST00000540670; ENSP00000441523; ENSG00000196365 [P36776-3] ENST00000593119; ENSP00000468541; ENSG00000196365 [P36776-2] |
GeneIDi | 9361 |
KEGGi | hsa:9361 |
UCSCi | uc002mcx.5, human [P36776-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
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 |
CCDSi | CCDS12148.1 [P36776-1] CCDS62507.1 [P36776-3] CCDS62508.1 [P36776-2] |
PIRi | S42366 S57342 |
RefSeqi | NP_001263408.1, NM_001276479.1 [P36776-2] NP_001263409.1, NM_001276480.1 [P36776-3] NP_004784.2, NM_004793.3 [P36776-1] |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
2X36 | X-ray | 2.00 | A/B/C/D/E/F | 753-959 | [»] | |
7KSL | electron microscopy | 3.40 | A/B/C/E/F | 421-947 | [»] | |
7KSM | electron microscopy | 3.20 | A/B/C/D/E/F | 416-947 | [»] | |
SMRi | P36776 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 114762, 291 interactors |
IntActi | P36776, 75 interactors |
MINTi | P36776 |
STRINGi | 9606.ENSP00000353826 |
Protein family/group databases
MEROPSi | S16.002 |
MoonDBi | P36776, Curated |
PTM databases
iPTMneti | P36776 |
MetOSitei | P36776 |
PhosphoSitePlusi | P36776 |
SwissPalmi | P36776 |
Genetic variation databases
BioMutai | LONP1 |
DMDMi | 12644239 |
Proteomic databases
EPDi | P36776 |
jPOSTi | P36776 |
MassIVEi | P36776 |
MaxQBi | P36776 |
PaxDbi | P36776 |
PeptideAtlasi | P36776 |
PRIDEi | P36776 |
ProteomicsDBi | 24918 55221 [P36776-1] |
Protocols and materials databases
Antibodypediai | 811, 285 antibodies |
DNASUi | 9361 |
Genome annotation databases
Ensembli | ENST00000360614; ENSP00000353826; ENSG00000196365 [P36776-1] ENST00000540670; ENSP00000441523; ENSG00000196365 [P36776-3] ENST00000593119; ENSP00000468541; ENSG00000196365 [P36776-2] |
GeneIDi | 9361 |
KEGGi | hsa:9361 |
UCSCi | uc002mcx.5, human [P36776-1] |
Organism-specific databases
CTDi | 9361 |
DisGeNETi | 9361 |
GeneCardsi | LONP1 |
HGNCi | HGNC:9479, LONP1 |
HPAi | ENSG00000196365, Tissue enhanced (adrenal) |
MalaCardsi | LONP1 |
MIMi | 600373, phenotype 605490, gene |
neXtProti | NX_P36776 |
OpenTargetsi | ENSG00000196365 |
Orphaneti | 1458, CODAS syndrome 79243, Pyruvate dehydrogenase E1-alpha deficiency |
PharmGKBi | PA162394145 |
VEuPathDBi | HostDB:ENSG00000196365.11 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG2004, Eukaryota |
GeneTreei | ENSGT00530000063553 |
HOGENOMi | CLU_004109_1_1_1 |
InParanoidi | P36776 |
OMAi | ENVAHED |
OrthoDBi | 528132at2759 |
PhylomeDBi | P36776 |
TreeFami | TF105001 |
Enzyme and pathway databases
BRENDAi | 3.4.21.53, 2681 |
PathwayCommonsi | P36776 |
Miscellaneous databases
BioGRID-ORCSi | 9361, 762 hits in 992 CRISPR screens |
ChiTaRSi | LONP1, human |
EvolutionaryTracei | P36776 |
GeneWikii | LONP1 |
GenomeRNAii | 9361 |
Pharosi | P36776, Tbio |
PROi | PR:P36776 |
RNActi | P36776, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000196365, Expressed in right adrenal gland and 233 other tissues |
ExpressionAtlasi | P36776, baseline and differential |
Genevisiblei | P36776, HS |
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 |
Pfami | View protein in Pfam PF00004, AAA, 1 hit PF05362, Lon_C, 1 hit PF02190, LON_substr_bdg, 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 |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | LONM_HUMAN | |
Accessioni | P36776Primary (citable) accession number: P36776 Secondary accession number(s): B3KPH8 Q9UQ95 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | June 1, 1994 |
Last sequence update: | December 1, 2000 | |
Last modified: | April 7, 2021 | |
This is version 204 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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, Reference proteomeDocuments
- Peptidase families
Classification of peptidase families and list of entries - Human chromosome 19
Human chromosome 19: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families