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Overview

Uniprot IDA0A5G2R1I5
Protein NameLon protease homolog, mitochondrial
Gene NameLONP1
OrganismSus scrofa

Kla Sites from experimental identification

Position Flanking peptide
328 KIKQTHRKYLLQEQL
513 GKIIQCLKKTKTENP
623 QCGLDESKAKLSSDV
76 PVFPRFIKIVEVKNK

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 RGPVITALTP 60 MTIPDVFPHL 70 PLIAVTRNPV 80 FPRFIKIVEV 90 KNKKLVELLR 100 RKVRLAQPYA 110 GVFLKRDDNN 120 ESDVVESLDE 130 IYHTGTFVQI 140 HEMQDLGDKL 150 RMIVMGHRRV 160 HINRQLEVEP 170 EEPEGEKQKP 180 RRKPKRSKKE 190 VEEDGGAKQQ 200 VEVVVEPGLS 210 PTGEVLMVEV 220 ENVVHEDFQV 230 TEEVKALTAE 240 IVKTIRDIIA 250 LNPLYRESVL 260 QMMQAGHRVV 270 DNPIYLSDMG 280 AALTGAESHE 290 LQDVLEETNI 300 PKRLYKALSL 310 LKKEFELSKL 320 QQRLGREVEE 330 KIKQTHRKYL 340 LQEQLKIIKK 350 ELGLEKDDKD 360 AIEEKFRERL 370 KELVVPKHVM 380 DVVDEELSKL 390 GLLDNHSSEF 400 NVTRNYLDWL 410 TSIPWGKYSD 420 ENLDLARAQA 430 VLEEDHYGME 440 DVKKRILEFI 450 AVSQLRGSTQ 460 GKILCFYGPP 470 GVGKTSIARS 480 IARALNREYF 490 RFSVGGMTDV 500 AEIKGHRRTY 510 VGAMPGKIIQ 520 CLKKTKTENP 530 LVLIDEVDKI 540 GRGYQGDPSS 550 ALLELLDPEQ 560 NANFLDHYLD 570 VPVDLSKVLF 580 ICTANITETI 590 PEPLRDRMEM 600 INVSGYVAQE 610 KLAIAERYLV 620 PQARAQCGLD 630 ESKAKLSSDV 640 LTLLIKQYCR 650 ESGVRNLQKQ 660 VEKVLRKSAY 670 KIVSGEAEFV 680 EVTPENLQDF 690 VGKPVFTVER 700 MYDVTPPGVV 710 MGLAWTAMGG 720 STLFVETSLR 730 RPRDRDSTKG 740 DKDGSLEVTG 750 QLGEVMKESA 760 RIAYTFARAF 770 LMQHDPTNEY 780 LVTSHIHLHV 790 PEGATPKDGP 800 SAGCTIVTAL 810 LSLAMDRPVR 820 QNLAMTGEVS 830 LTGKVLPVGG 840 IKEKTIAAKR 850 AGVTCIVLPA 860 ENKKDFYDLA 870 AFITEGLEVH 880 FVEHYREIFD 890 IAFPEEQAEA LAVER

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.