Page 167 - 《南京医科大学学报》2026年第1期
P. 167

第46卷第1期                    张晓璐,李杉姗,徐 颖. LON蛋白酶1在疾病中的研究进展[J].
                  2026年1月                      南京医科大学学报(自然科学版),2026,46(1):153-162                      · 161  ·


               [28]TANG P,ZENG Q,LI Y H,et al. The mitochondrial       ticancer therapeutics[J]. Expert Opin Ther Targets,
                    LONP1 protease:molecular targets and role in pathophysi⁃  2024,28(1⁃2):9-15
                    ology[J]. Mol Biol Rep,2025,52(1):401        [42]GHOSH J C,SEO J H,AGARWAL E,et al. Akt phosphory⁃
               [29]ZANINI G,SELLERI V,MALERBA M,et al. The role of     lation of mitochondrial Lonp1 protease enables oxidative
                    Lonp1 on mitochondrial functions during cardiovascular  metabolism and advanced tumor traits[J]. Oncogene,
                    and muscular diseases[J]. Antioxidants(Basel),2023,12  2019,38(43):6926-6939
                   (3):598                                       [43]GIBELLINI L,LOSI L,DE BIASI S,et al. LonP1 differ⁃
               [30]KIM Y,TRIOLO M,HOOD D A. Impact of aging and        ently modulates mitochondrial function and bioenergetics
                    exercise on mitochondrial quality control in skeletal mus⁃  of primary versus metastatic colon cancer cells[J]. Front
                    cle[J]. Oxid Med Cell Longev,2017,2017:3165396 ⁃   Oncol,2018,8:254
                    3165396                                      [44]LIU C,WANG H,LI H,et al. Inhibition of LONP1 sup⁃
               [31]DELAVAL E,PERICHON M,FRIGUET B. Age⁃related         presses pancreatic cancer progression via c⁃Jun N⁃Termi⁃
                    impairment of mitochondrial matrix aconitase and ATP ⁃  nal kinase pathway ⁃ meditated epithelial ⁃ mesenchymal
                    stimulated protease in rat liver and heart[J]. Eur J Bio⁃  transition[J]. Pancreas,2019,48(5):629-635
                    chem,2004,271(22):4559-4564                  [45]WANG X N,XU X,ZHAO Y,et al. Inhibition of Lonp1 in⁃
               [32]ZHU W,TAN C,ZHANG J. Aging of alveolar type 2 cells  duces mitochondrial remodeling and autophagy suppres⁃
                    induced by LONP1 deficiency exacerbates pulmonary fi⁃  sion in cervical cancer cells[J]. Acta Histochemical,
                    brosis[J]. Biomol Biomed,2024,24(5):1258-1272      2023,125(1):151986
               [33]ZHANG C,SHEN S,XU L,et al. LONP1 alleviates ageing  [46]LUO B,WANG M G,HOU N Y,et al. ATP⁃dependent
                    ⁃ related renal fibrosis by maintaining mitochondrial ho⁃  LON protease contributes to helicobacter pylori⁃induced
                    meostasis[J]. J Cell Mol Med,2024,28(17):e70090    gastric carcinogenesis[J]. Neoplasia(NY),2016,18(4):
               [34]FU T,SUN W,XUE J et al. Proteolytic rewiring of mito⁃  242-252
                    chondria by LONP1 directs cell identity switching of adi⁃  [47]ZHONG Y,WU L,PENG Z,et al. LONP1 Promotes hepa⁃
                    pocytes[J]. Nat Cell Biol,2023,25(6):848-864       tocarcinogenesis by degrading ACO2 to alleviate ferropto⁃
               [35]TANG Y,LIU Y X,SHENG Y,et al. The first case report  sis[J]. Front Biosci(Landmark Ed),2025,30(8):41226
                    of CODAS syndrome in Chinese population caused by two  [48]YAO M,ZHANG X,WU T,et al. LON protease 1⁃mediat⁃
                    LONP1 pathogenic mutations[J]. Front Genet,2023,13:  ed metabolic reprogramming promotes the progression of

                    1031856                                            prostate cancer[J]. Cell Death Dis,2025,16(1):116
               [36]NIMMO G A M,VENKATESH S,PANDEY A K,et al. Bi⁃  [49]BERNSTEIN S H,VENKATESH S,LI M,et al. The mito⁃
                    allelic mutations of LONP1 encoding the mitochondrial  chondrial ATP⁃dependent LON protease:a novel target in
                    LonP1 protease cause pyruvate dehydrogenase deficiency  lymphoma death mediated by the synthetic triterpenoid
                    and profound neurodegeneration with progressive cerebel⁃  CDDO and its derivatives[J]. Blood,2012,119(14):
                    lar atrophy[J]. Hum Mol Genet,2019,28(2):290-306   3321-3329
               [37]LI S,HE N,LIAO J,et al. Association of LONP1 gene  [50]MANEIX L,SWEENEY M A,LEE S,et al. The mitochon⁃
                    with epilepsy and the sub ⁃ regional effect[J]. Sci Rep,  drial protease LONP1 promotes proteasome inhibitor re⁃
                    2024,14(1):25575                                   sistance in multiple myeloma[J]. Cancers,2021,13(4):
               [38]PETER B,WADDINGTON C L,OLÁHOVÁ M,et al. De⁃         843
                    fective mitochondrial protease LonP1 can cause classical  [51]MUTHU S,TRAN Z,THILAGAVATHI J,et al. Aging trig⁃
                    mitochondrial disease[J]. Hum Mol Genet,2018,27    gers mitochondrial,endoplasmic reticulum,and metabolic
                   (10):1743-1753                                      stress responses in the heart[J]. J Cardiovasc Aging,
               [39]HANNAH⁃SHMOUNI F,MACNEIL L,BRADY L,et al.           2025,5(1):4
                    Expanding the clinical spectrum of LONP1⁃related mito⁃  [52]ZHAO K,HUANG X Y,ZHAO W K,et al. LONP1⁃medi⁃
                    chondrial cytopathy[J]. Front Neurol,2019,10:981   ated mitochondrial quality control safeguards metabolic
               [40]QIAO L,XU L,YU L,et al. Rare and de novo variants in  shifts in heart development[J]. Development,2022,149
                    827 congenital diaphragmatic hernia probands implicate  (6):dev200458
                    LONP1 as candidate risk gene[J]. Am J Hum Genet,  [53]SEPURI N B V,ANGIREDDY R,SRINIVASAN S,et al.
                    2021,108(10):1964-1980                             Mitochondrial LON protease⁃dependent degradation of cy⁃
               [41]SHETTY R,NOLAND R,NANDI G,et al. Powering down      tochrome C oxidase subunits under hypoxia and myocardi⁃
                    the mitochondrial LONP1 protease:a novel strategy for an⁃  al ischemia[J]. Biochim Biophys Acta Bioenerg,2017,
   162   163   164   165   166   167   168   169   170