Search Results

Overview

Uniprot IDP36776
Protein NameLon protease homolog, mitochondrial
Gene NameLONP1
OrganismHomo sapiens

Kla Sites from experimental identification

Position Flanking peptide
244 RKSKRGKKEAEDELS
374 KKEFELSKLQQRLGR
393 KIKQTHRKYLLQEQL
426 EKFRERLKELVVPKH
432 LKELVVPKHVMDVVD
572 YVGAMPGKIIQCLKK
898 PVGGIKEKTIAAKRA

Function

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 (PubMed:12198491, PubMed:15870080, PubMed:17579211, PubMed:37327776, PubMed:8248235, PubMed:35870450, PubMed:38688959). Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, DELE1, helicase Twinkle (TWNK) and the large ribosomal subunit protein MRPL32/bL32m (PubMed:17579211, PubMed:28377575, PubMed:37327776). MRPL32/bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not (PubMed:17579211, PubMed:28377575). In addtion to its protease activity, has also an ATP-dependent protein folding chaperone activity and functions in mitochondrial protein folding (PubMed:33431889). Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome (PubMed:17420247). Binds to mitochondrial promoters and RNA in a single-stranded, site-specific, and strand-specific manner (PubMed:17420247). 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 (PubMed:14739292, PubMed:17420247)

Protein Sequence

10 MAASTGYVRL 20 WGAARCWVLR 30 RPMLAAAGGR 40 VPTAAGAWLL 50 RGQRTCDASP 60 PWALWGRGPA 70 IGGQWRGFWE 80 ASSRGGGAFS 90 GGEDASEGGA 100 EEGAGGAGGS 110 AGAGEGPVIT 120 ALTPMTIPDV 130 FPHLPLIAIT 140 RNPVFPRFIK 150 IIEVKNKKLV 160 ELLRRKVRLA 170 QPYVGVFLKR 180 DDSNESDVVE 190 SLDEIYHTGT 200 FAQIHEMQDL 210 GDKLRMIVMG 220 HRRVHISRQL 230 EVEPEEPEAE 240 NKHKPRRKSK 250 RGKKEAEDEL 260 SARHPAELAM 270 EPTPELPAEV 280 LMVEVENVVH 290 EDFQVTEEVK 300 ALTAEIVKTI 310 RDIIALNPLY 320 RESVLQMMQA 330 GQRVVDNPIY 340 LSDMGAALTG 350 AESHELQDVL 360 EETNIPKRLY 370 KALSLLKKEF 380 ELSKLQQRLG 390 REVEEKIKQT 400 HRKYLLQEQL 410 KIIKKELGLE 420 KDDKDAIEEK 430 FRERLKELVV 440 PKHVMDVVDE 450 ELSKLGLLDN 460 HSSEFNVTRN 470 YLDWLTSIPW 480 GKYSNENLDL 490 ARAQAVLEED 500 HYGMEDVKKR 510 ILEFIAVSQL 520 RGSTQGKILC 530 FYGPPGVGKT 540 SIARSIARAL 550 NREYFRFSVG 560 GMTDVAEIKG 570 HRRTYVGAMP 580 GKIIQCLKKT 590 KTENPLILID 600 EVDKIGRGYQ 610 GDPSSALLEL 620 LDPEQNANFL 630 DHYLDVPVDL 640 SKVLFICTAN 650 VTDTIPEPLR 660 DRMEMINVSG 670 YVAQEKLAIA 680 ERYLVPQARA 690 LCGLDESKAK 700 LSSDVLTLLI 710 KQYCRESGVR 720 NLQKQVEKVL 730 RKSAYKIVSG 740 EAESVEVTPE 750 NLQDFVGKPV 760 FTVERMYDVT 770 PPGVVMGLAW 780 TAMGGSTLFV 790 ETSLRRPQDK 800 DAKGDKDGSL 810 EVTGQLGEVM 820 KESARIAYTF 830 ARAFLMQHAP 840 ANDYLVTSHI 850 HLHVPEGATP 860 KDGPSAGCTI 870 VTALLSLAMG 880 RPVRQNLAMT 890 GEVSLTGKIL 900 PVGGIKEKTI 910 AAKRAGVTCI 920 VLPAENKKDF 930 YDLAAFITEG 940 LEVHFVEHYR 950 EIFDIAFPDE QAEALAVER

Gene Ontology

Classification GO ID Description
Cellular Component GO:0016020 membrane
Cellular Component GO:0005759 mitochondrial matrix
Cellular Component GO:0042645 mitochondrial nucleoid
Cellular Component GO:0005739 mitochondrion
Molecular Function GO:0043531 ADP binding
Molecular Function GO:0005524 ATP binding
Molecular Function GO:0016887 ATP hydrolysis activity
Molecular Function GO:0004176 ATP-dependent peptidase activity
Molecular Function GO:0070182 DNA polymerase binding
Molecular Function GO:0051880 G-quadruplex DNA binding
Molecular Function GO:0042802 identical protein binding
Molecular Function GO:0043560 insulin receptor substrate binding
Molecular Function GO:0042731 PH domain binding
Molecular Function GO:0043565 sequence-specific DNA binding
Molecular Function GO:0004252 serine-type endopeptidase activity
Molecular Function GO:0003697 single-stranded DNA binding
Molecular Function GO:0003727 single-stranded RNA binding
Biological Process GO:0034599 cellular response to oxidative stress
Biological Process GO:0051131 chaperone-mediated protein complex assembly
Biological Process GO:0035694 mitochondrial protein catabolic process
Biological Process GO:0007005 mitochondrion organization
Biological Process GO:0070407 oxidation-dependent protein catabolic process
Biological Process GO:0006515 protein quality control for misfolded or incompletely synthesized proteins
Biological Process GO:0051603 proteolysis involved in protein catabolic process
Biological Process GO:0010044 response to aluminum ion
Biological Process GO:0009725 response to hormone
Biological Process GO:0001666 response to hypoxia

Reference

[1] Yang Z, Yan C, Ma J, Peng P, Ren X et al.. Lactylome analysis suggests lactylation-dependent mechanisms of metabolic adaptation in hepatocellular carcinoma.. Nat Metab 5(1):61-79. 2023 Jan. PMID: 36593272.

[2] Hong H, Chen X, Wang H, Gu X, Yuan Y et al.. Global profiling of protein lysine lactylation and potential target modified protein analysis in hepatocellular carcinoma.. Proteomics 23(9):e2200432. 2023 May. PMID: 36625413.

[3] Shi CM, Wang QC, Li XL, Yang YH, Tang XY et al.. Global Profiling of Protein Lactylation in Human Hippocampi.. Proteomics Clin Appl 19(2):e202400061. 2025 Mar. PMID: 39610256.