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Overview

Uniprot IDF1SBU7
Protein NameLon protease homolog, mitochondrial
Gene NameLONP1
OrganismSus scrofa

Kla Sites from experimental identification

Position Flanking peptide
141 PVFPRFIKIVEVKNK
365 KIKQTHRKYLLQEQL
550 GKIIQCLKKTKTENP
660 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 KIVEVKNKKL 160 VELLRRKVRL 170 AQPYAGVFLK 180 RDDNNESDVV 190 ESLDEIYHTG 200 TFVQIHEMQD 210 LGDKLRMIVM 220 GHRRVHINRQ 230 LEVEPEEPEG 240 EKQKPRRKPK 250 RSKKEVEEDG 260 GAKQQVEVVV 270 EPGLSPTGEV 280 LMVEVENVVH 290 EDFQVTEEVK 300 ALTAEIVKTI 310 RDIIALNPLY 320 RESVLQMMQA 330 GHRVVDNPIY 340 LSDMGAALTG 350 AESHELQDVL 360 EETNVEEKIK 370 QTHRKYLLQE 380 QLKIIKKELG 390 LEKDDKDAIE 400 EKFRERLKEL 410 VVPKHVMDVV 420 DEELSKLGLL 430 DNHSSEFNVT 440 RNYLDWLTSI 450 PWGKYSDENL 460 DLARAQAVLE 470 EDHYGMEDVK 480 KRILEFIAVS 490 QLRGSTQGKI 500 LCFYGPPGVG 510 KTSIARSIAR 520 ALNREYFRFS 530 VGGMTDVAEI 540 KGHRRTYVGA 550 MPGKIIQCLK 560 KTKTENPLVL 570 IDEVDKIGRG 580 YQGDPSSALL 590 ELLDPEQNAN 600 FLDHYLDVPV 610 DLSKVLFICT 620 ANITETIPEP 630 LRDRMEMINV 640 SGYVAQEKLA 650 IAERYLVPQA 660 RAQCGLDESK 670 AKLSSDVLTL 680 LIKQYCRESG 690 VRNLQKQVEK 700 VLRKSAYKIV 710 SGEAEFVEVT 720 PENLQDFVGK 730 PVFTVERMYD 740 VTPPGVVMGL 750 AWTAMGGSTL 760 FVETSLRRPR 770 DRDSTKGDKD 780 GSLEVTGQLG 790 EVMKESARIA 800 YTFARAFLMQ 810 HDPTNEYLVT 820 SHIHLHVPEG 830 ATPKDGPSAG 840 CTIVTALLSL 850 AMDRPVRQNL 860 AMTGEVSLTG 870 KVLPVGGIKE 880 KTIAAKRAGV 890 TCIVLPAENK 900 KDFYDLAAFI 910 TEGLEVHFVE 920 HYREIFDIAF 930 PEEQAEALAV ER

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.