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Overview

Uniprot IDA0A5G2R1B2
Protein NameLon protease homolog, mitochondrial
Gene NameLONP1
OrganismSus scrofa

Kla Sites from experimental identification

Position Flanking peptide
141 PVFPRFIKIVEVKAG
383 KIKQTHRKYLLQEQL
568 GKIIQCLKKTKTENP
678 QCGLDESKAKLSSDV

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. 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

Protein Sequence

10 MAAGAGCVRL 20 WGAARWWALR 30 RPLLAAAAGR 40 VPTAVRAWLP 50 RGRRACDASP 60 PWAQWGQGPA 70 AAGHWRGLWE 80 ANNRSSGGAF 90 SGGEDAPEGG 100 AEDGASGVGG 110 NAGGGEGPVI 120 TALTPMTIPD 130 VFPHLPLIAV 140 TRNPVFPRFI 150 KIVEVKAGGC 160 VSPYPREPEC 170 LCPCSFVSLS 180 LSNESDVVES 190 LDEIYHTGTF 200 VQIHEMQDLG 210 DKLRMIVMGH 220 RRVHINRQLE 230 VEPEEPEGEK 240 QKPRRKPKRS 250 KKEVEEDGGA 260 KQQVEVVVEP 270 GLSPTGEVLM 280 VEVENVVHED 290 FQVTEEVKVL 300 APHGSISVPS 310 RESVLQMMQA 320 GHRVVDNPIY 330 LSDMGAALTG 340 AESHELQDVL 350 EETNIPKRLY 360 KALSLLKKEF 370 ELSKLQQRLG 380 REVEEKIKQT 390 HRKYLLQEQL 400 KIIKKELGLE 410 KDDKDAIEEK 420 FRERLKELVV 430 PKHVMDVVDE 440 ELSKLGLLDN 450 HSSEFNVTRN 460 YLDWLTSIPW 470 GKYSDENLDL 480 ARAQAVLEED 490 HYGMEDVKKR 500 ILEFIAVSQL 510 RGSTQGKILC 520 FYGPPGVGKT 530 SIARSIARAL 540 NREYFRFSVG 550 GMTDVAEIKG 560 HRRTYVGAMP 570 GKIIQCLKKT 580 KTENPLVLID 590 EVDKIGRGYQ 600 GDPSSALLEL 610 LDPEQNANFL 620 DHYLDVPVDL 630 SKVLFICTAN 640 ITETIPEPLR 650 DRMEMINVSG 660 YVAQEKLAIA 670 ERYLVPQARA 680 QCGLDESKAK 690 LSSDVLTLLI 700 KQYCRESGVR 710 NLQKQVEKVL 720 RKSAYKIVSG 730 EAEFVEVTPE 740 NLQDFVGKPV 750 FTVERMYDVT 760 PPGVVMGLAW 770 TAMGGSTLFV 780 ETSLRRPRDR 790 DSTKGDKDGS 800 LEVTGQLGEV 810 MKESARIAYT 820 FARAFLMQHD 830 PTNEYLVTSH 840 IHLHVPEGAT 850 PKDGPSAGCT 860 IVTALLSLAM 870 DRPVRQNLAM 880 TGEVSLTGKV 890 LPVGGIKEKT 900 IAAKRAGVTC 910 IVLPAENKKD 920 FYDLAAFITE 930 GLEVHFVEHY 940 REIFDIAFPE 950 EQAEALAVER

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005759 mitochondrial matrix
Molecular Function GO:0005524 ATP binding
Molecular Function GO:0016887 ATP hydrolysis activity
Molecular Function GO:0004176 ATP-dependent peptidase activity
Molecular Function GO:0043565 sequence-specific DNA binding
Molecular Function GO:0004252 serine-type endopeptidase activity
Biological Process GO:0034599 cellular response to oxidative stress
Biological Process GO:0051131 chaperone-mediated protein complex assembly
Biological Process GO:0070407 oxidation-dependent protein catabolic process
Biological Process GO:0006515 protein quality control for misfolded or incompletely synthesized proteins

Reference

[1] Fan S, Zhou R, Wen H, Ye H, Ma S et al.. Global profiling of protein lactylome in porcine granulosa cells.. J Ovarian Res 18(1):177. 2025 Aug 8. PMID: 40781717.