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              · 160  ·                           南 京    医 科 大 学 学         报                        2026年1月


                   in the apo ⁃ and substrate ⁃ bound states reveals a unique  dence for mitochondrial Lonp1 expression in the nu⁃
                   mode of action driven by oligomerization[J]. PLoS Biol,  cleus[J]. Sci Rep,2022,12(1):10877

                   2023,21(2):e3001987                          [15]WLODAWER A,SEKULA B,GUSTCHINA A,et al.
             [3] VENKATESH S,SUZUKI C K. Cell stress management      Structure and the mode of activity of lon proteases from di⁃
                   by the mitochondrial LonP1 protease ⁃ insights into miti⁃  verse organisms[J]. J Mol Biol,2022,434(7):167504
                   gating developmental,oncogenic and cardiac stress[J].  [16]SHIN M,WATSON E R,SONG A S,et al. Structures of
                   Mitochondrion,2020,51:46-61                       the human LONP1 protease reveal regulatory steps in⁃
             [4] KIRTHIKA P,LLOREN K K S,JAWALAGATTI V,et al.        volved in protease activation[J]. Nat Commun,2021,12
                   Structure,substrate specificity and role of lon protease in  (1):3239
                   bacterial pathogenesis and survival[J]. Int J Mol Sci,  [17]MINDREBO J T,LANDER G C. Structural and mechanis⁃
                   2023,24(4):3422                                   tic studies on human LONP1 redefine the hand⁃over⁃hand
             [5] YIGIT K,CHIEN P. LON⁃dependent proteolysis in oxida⁃  translocation mechanism[J]. bioRxiv,2024,doi:10.1101/
                   tive stress responses[J].J Bacteriol,2025,207(7):  2024.06.24.600538
                   e00005-00025                                 [18]肖   磊,潘佳滢. 线粒体Lon蛋白酶的生理作用研究进
             [6] GIBELLINI L,DE GAETANO A,MANDRIOLI M,et al.         展[J]. 生物化工,2021,7(5):155-157
                   The biology of Lonp1:more than a mitochondrial prote⁃  XIAO L,PAN J Y. Research progress on the physiologi⁃
                   ase[J]. Int. Rev. Cell Mol. Biol,2020,354:1-61    cal function of mitochondrial lon protease[J]. Biological
             [7] XI W W,ZHANG Z Y,SU Y,et al. Frontiers in LONP1     Chemical Engineering,2021,7(5):155-157
                   research:unraveling molecular mechanisms,disease asso⁃  [19]POLLECKER K,SYLVESTER M,VOOS W. Proteomic
                   ciations,and therapeutic strategies[J]. Mitochondrial  analysis demonstrates the role of the quality control prote⁃
                   Commun,2025,3:79-86                               ase LONP1 in mitochondrial protein aggregation[J]. J Bi⁃
             [8] 渠允薇,张世纪,张铎腾,等. 线粒体蛋白酶 LONP1 结                       ol Chem,2021,297(4):101134
                   构、功能与相关疾病研究进展[J]. 江苏大学学报(医学                  [20]ZANINI G,MICHELONI G,SINIGAGLIA G,et al. Modu⁃
                   版),2024,34(4):354-363                             lation of Lonp1 activity by small compounds[J]. Biomole⁃
                   QU Y W,ZHANG S J,ZHANG D T,et al. Research prog⁃  cules,2025,15(4):553
                   ress on structure,function and related diseases of mito⁃  [21]TANG L,YU R,ZHOU X S,et al. Mitochondria transcrip⁃
                   chondrial protease LONP1[J]. Journal of Jiangsu Univer⁃  tion and cancer[J]. Cell Death Discov,2024,10(1):168
                   sity(Medical Edition),2024,34(4):354-363     [22]LONG Q,ZHOU Y S,WU H,et al. Phase separation
             [9] CUI X B,ZHANG Y L,ZHANG L,et al. Role of LONP2      drives the self ⁃ assembly of mitochondrial nucleoids for
                   in head and neck squamous cell carcinoma[J]. Gene,  transcriptional modulation[J]. Nat Struct Mol Biol,2021,
                   2023,851:147033                                   28(11):900-908
             [10]WANG J L,ZHANG Y,SHI H J,et al. Construction of mi⁃  [23]YANG Q Y,LIU P P,ANDERSON N S,et al. LONP⁃1
                   tochondrial protection and monitoring model of lon prote⁃  and ATFS⁃1 sustain deleterious heteroplasmy by promot⁃
                   ase based on machine learning under myocardial isch⁃  ing mtDNA replication in dysfunctional mitochondria[J].
                   emia environment[J]. J Environ Public Health,2022,  Nat Cell Biol,2022,24(2):181-193
                   2022:4805009                                 [24]BRADLEY J,SWANN K. Mitochondria and lipid metabo⁃
             [11]MATSUSHIMA Y,TAKAHASHI K,YUE S,et al. Mito⁃         lism in mammalian oocytes and early embryos[J]. Int J
                   chondrial LON protease is a gatekeeper for proteins newly  Dev Biol,2019,63(3⁃4⁃5):93-103
                   imported into the matrix[J]. Commun Biol,2021,4(1):  [25]SHENG X Q,LIU C M,YAN G J,et al. The mitochondri⁃
                   974                                               al protease LONP1 maintains oocyte development and sur⁃
             [12]BESSE A,BREZAVAR D,HANSON J,et al. LONP1 de         vival by suppressing nuclear translocation of AIFM1 in
                   novo dominant mutation causes mitochondrial encepha⁃  mammals[J]. EBioMedicine,2022,75:103790
                   lopathy with loss of LONP1 chaperone activity and exces⁃  [26]LIU C M,XU M L,GUAN Y J,et al. Decreased LONP1
                   sive LONP1 proteolytic activity[J]. Mitochondrion,2020,  expression contributes to DNA damage and meiotic de⁃

                   51:68-78                                          fects in oocytes[J]. Mol Reprod Dev,2023,90(6):358-368
             [13]LI Y J,HUANG D W,JIA L Q,et al. LonP1 links mito⁃  [27]STRAUSS K A,JINKS R N,PUFFENBERGER E G,et al.
                   chondria ⁃ ER interaction to regulate heart function[J].  CODAS syndrome is associated with mutations of LONP1,
                   Research(Wash D C),2023,6:0175                    encoding mitochondrial AAA + Lon protease[J]. Am J
             [14]GIBELLINI L,BORELLA R,DE GAETANO A,et al. Evi⁃      Hum Genet,2015,96(1):121-135
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