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Literature for peptidase S16.002: PIM1 peptidase

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Structure Literature Substrates

(Topics flags: S Structure, A Assay, T Target, P Specificity, K Knockout, V Review, M Mutation, I Inhibitor. To select only the references relevant to a single topic, click the link above. See explanation.)

    2026
  1. Ali,L., Mallick,A., Du,Y., Li,R., Zhu,L.J., Shen,K. and Haynes,C.M.<br>LONP-1 deficiency causes dysregulated protein synthesis within mitochondria that is restored by mitoribosomal mutations<br>bioRxiv (2026) PubMed  Europe PubMed DOI  PMC  EPMC
    2. 2025
  2. Boggula,Y., Chatterjee,A., Simaiya,G., Pal,A., Srinivasan,A. and Sepuri,N.B.<br>Lon/Pim1 mediated degradation of presequence translocase-associated motor components Pam16 and Pam18 in Saccharomyces cerevisiae<br>Biochem J (2025) PubMed  Europe PubMed DOI
  3. Li,J., Deng,Y., Gasser,M., Zhu,J., Walker,E.M., Sidarala,V., Reck,E.C., Hubers,D.L., Pasmooij,M.B., Shin,C.S., Bandesh,K., Motakis,E., Nargund,S., Kursawe,R., Basrur,V., Nesvizhskii,A.I., Stitzel,M.L., Chan,D.C., Rutter,G.A. and Soleimanpour,S.A.<br>LONP1 regulation of mitochondrial protein folding provides insight into beta cell failure in type 2 diabetes<br>Nat Metab (2025) PubMed  Europe PubMed DOI
  4. Llanquinao-Sandoval,J., Cicali,K.A., Jara,C., Vigil-Vasquez,C., Sjoberg-Herrera,M.K., Ricca,M., Valenzuela,S., Loyola,A., Pinti,M., Schuller,A., Kerr,B. and Tapia-Rojas,C.<br>Identifying a novel Mecp2-mediated epigenetic mechanism controlling Lonp1 in the hippocampus and its disruption by aging<br>Sci Rep (2025) 15, 40887-40887. PubMed  Europe PubMed DOI  PMC  EPMC
  5. Mindrebo,J.T. and Lander,G.C.<br>Substrate-dependent activation of LONP1 informs on proteolytic regulation and ATPase motor function<br>Proc Natl Acad Sci U S A (2025) 122, e2415153122-e2415153122. PubMed  Europe PubMed DOI  PMC  EPMC
  6. Wu,D., Zhang,S., Wu,Y., Bi,X., Zhou,Y. and Wu,W.<br>Sulforaphane downregulates mitochondrial TIGAR via inhibiting mitochondrial transmembrane assembly and LONP1/CASP3 axis causing apoptosis<br>Biochem Biophys Res Commun (2025) 760, 151689-151689. PubMed  Europe PubMed DOI  K
  7. Young,R.E., Qiao,L., Hernan,R., Sweetser,D.A., Waxler,J.L., Scott,D.A., Scott,T.M., Lalani,S.R., Azamian,M.S., Rosenfeld,J.A., Bostwick,B., Burrage,L.C., Rodan,L.H., Russell,B.E., Dutra-Clarke,M., Kruer,M., Bakhtiarim,S., Darvish,H., Amor,D.J., Rahman,S., Stals,K., Bradley,L., Byrne,S., Tolusso,L.K., Wong,B., Benedict,L., Wallis,K., Micke,K., Colson,C., Smol,T., Southwick,S.V., Miller,K.A., Kush,M.L., Chorin,O., Rothschild,A., Wang,W., Shen,Y. and Chung,W.K.<br>LONP1 Variants Are Associated With Clinically Diverse Phenotypes<br>Clin Genet (2025) PubMed  Europe PubMed DOI
  8. Zhong,Y., Wu,L., Peng,Z., Mao,W., Wang,T., Wu,B., Yu,Z., Li,X. and Chen,E.<br>LONP1 Promotes Hepatocarcinogenesis by Degrading ACO2 to Alleviate Ferroptosis<br>Front Biosci (Landmark Ed) (2025) 30, 41226-41226. PubMed  Europe PubMed DOI  K
    9. 2024
  9. Liu,Y., Jiang,J.J., Du,S.Y., Mu,L.S., Fan,J.J., Hu,J.C., Ye,Y., Ding,M., Zhou,W.Y., Yu,Q.H., Xia,Y.F., Xu,H.Y., Shi,Y.J., Qian,S.W., Tang,Y., Li,W., Dang,Y.J., Dong,X., Li,X.Y., Xu,C.J. and Tang,Q.Q.<br>Artemisinins ameliorate polycystic ovarian syndrome by mediating LONP1-CYP11A1 interaction<br>Science (2024) 384, eadk5382-eadk5382. PubMed  Europe PubMed DOI  I
  10. Shetty,R., Noland,R., Nandi,G. and Suzuki,C.K.<br>Powering down the mitochondrial LonP1 protease: a novel strategy for anticancer therapeutics<br>Expert Opin Ther Targets (2024) 28, 9-15. PubMed  Europe PubMed DOI  PMC  EPMC  V
  11. Su,X.L., Su,Z.R. and Xu,W.H.<br>The protease Lon prolongs insect lifespan by responding to reactive oxygen species and degrading mitochondrial transcription factor A<br>Biochim Biophys Acta Mol Cell Res (2024) 1871, 119648-119648. PubMed  Europe PubMed DOI
  12. Zhang,C., Shen,S., Xu,L., Li,M., Tian,B., Yao,L. and Zhu,X.<br>LONP1 alleviates ageing-related renal fibrosis by maintaining mitochondrial homeostasis<br>J Cell Mol Med (2024) 28, e70090-e70090. PubMed  Europe PubMed DOI  PMC  EPMC
    13. 2023
  13. Douglas,C., Lomeli,N., Lepe,J., Di,K., Nandwana,N.K., Vu,T., Pham,J., Kenney,M.C., Das,B. and Bota,D.A.<br>Discovery and Validation of Novel LonP1 and Proteasome Inhibitor in IDH1-R132H Malignant Astrocytoma Models<br>bioRxiv (2023) PubMed  Europe PubMed DOI  PMC  EPMC  I
  14. Fu,T., Sun,W., Xue,J., Zhou,Z., Wang,W., Guo,Q., Chen,X., Zhou,D., Xu,Z., Liu,L., Xiao,L., Mao,Y., Yang,L., Yin,Y., Zhang,X.N., Wan,Q., Lu,B., Chen,Y., Zhu,M.S., Scherer,P.E., Fang,L., Piao,H.L., Shao,M. and Gan,Z.<br>Proteolytic rewiring of mitochondria by LONP1 directs cell identity switching of adipocytes<br>Nat Cell Biol (2023) 25, 848-864. PubMed  Europe PubMed DOI
  15. Li,Y., Huang,D., Jia,L., Shangguan,F., Gong,S., Lan,L., Song,Z., Xu,J., Yan,C., Chen,T., Tan,Y., Liu,Y., Huang,X., Suzuki,C.K., Yang,Z., Yang,G. and Lu,B.<br>LonP1 Links Mitochondria-ER Interaction to Regulate Heart Function<br>Research (Wash D C) (2023) 6, 0175-0175. PubMed  Europe PubMed DOI  PMC  EPMC
  16. Metzger,M.B., Scales,J.L., Grant,G.A., Molnar,A.E., Loncarek,J. and Weissman,A.M.<br>Differential sensitivity of the yeast Lon protease Pim1p to impaired mitochondrial respiration<br>J Biol Chem (2023) 299, 104937-104937. PubMed  Europe PubMed DOI  PMC  EPMC
  17. Wang,X., Xu,X., Zhao,Y., Qi,L. and Ge,H.<br>Inhibition of Lonp1 induces mitochondrial remodeling and autophagy suppression in cervical cancer cells<br>Acta Histochem (2023) 125, 151986-151986. PubMed  Europe PubMed DOI  K
  18. Xie,Y., Chen,S., Guo,Z., Tian,Y., Hong,X., Feng,P., Xie,Q. and Yu,Q.<br>Down-regulation of Lon protease 1 lysine crotonylation aggravates mitochondrial dysfunction in polycystic ovary syndrome<br>MedComm (2020) (2023) 4, e396-e396. PubMed  Europe PubMed DOI  PMC  EPMC
  19. Zanini,G., Selleri,V., Malerba,M., Solodka,K., Sinigaglia,G., Nasi,M., Mattioli,A.V. and Pinti,M.<br>The Role of Lonp1 on Mitochondrial Functions during Cardiovascular and Muscular Diseases<br>Antioxidants (Basel) (2023) 12 PubMed  Europe PubMed DOI  PMC  EPMC
    20. 2022
  20. Gibellini,L., Borella,R., De Gaetano,A., Zanini,G., Tartaro,D.L., Carnevale,G., Beretti,F., Losi,L., De Biasi,S., Nasi,M., Forcato,M., Cossarizza,A. and Pinti,M.<br>Evidence for mitochondrial Lonp1 expression in the nucleus<br>Sci Rep (2022) 12, 10877-10877. PubMed  Europe PubMed DOI  PMC  EPMC
  21. Hackett,P.T., Jia,X., Li,L. and Ward,D.M.<br>Posttranslational regulation of mitochondrial frataxin and identification of compounds that increase frataxin levels in Friedreich's ataxia<br>J Biol Chem (2022) 298, 101982-101982. PubMed  Europe PubMed DOI
  22. He,Y., Ding,Q., Chen,W., Lin,C., Ge,L., Ying,C., Xu,K., Wu,Z., Xu,L., Ran,J., Chen,W. and Wu,L.<br>LONP1 downregulation with ageing contributes to osteoarthritis via mitochondrial dysfunction<br>Free Radic Biol Med (2022) 191, 176-190. PubMed  Europe PubMed DOI  K  I
  23. Mohammed,I., Schmitz,K.A., Schenck,N., Balasopoulos,D., Topitsch,A., Maier,T. and Abrahams,J.P.<br>Catalytic cycling of human mitochondrial Lon protease<br>Structure (2022) 30, 1254-1268. PubMed  Europe PubMed DOI
  24. Sheng,X., Liu,C., Yan,G., Li,G., Liu,J., Yang,Y., Li,S., Li,Z., Zhou,J., Zhen,X., Zhang,Y., Diao,Z., Hu,Y., Fu,C., Yao,B., Li,C., Cao,Y., Lu,B., Yang,Z., Qin,Y., Sun,H. and Ding,L.<br>The mitochondrial protease LONP1 maintains oocyte development and survival by suppressing nuclear translocation of AIFM1 in mammals<br>EBioMedicine (2022) 75, 103790-103790. PubMed  Europe PubMed DOI  PMC  EPMC
  25. Taouktsi,E., Kyriakou,E., Smyrniotis,S., Borbolis,F., Bondi,L., Avgeris,S., Trigazis,E., Rigas,S., Voutsinas,G.E. and Syntichaki,P.<br>Organismal and Cellular Stress Responses upon Disruption of Mitochondrial Lonp1 Protease<br>Cells (2022) 11 PubMed  Europe PubMed DOI  PMC  EPMC  K  I
  26. Yang,J., Song,A.S., Wiseman,R.L. and Lander,G.C.<br>Cryo-EM structure of hexameric yeast Lon protease (PIM1) highlights the importance of conserved structural elements<br>J Biol Chem (2022) 298, 101694-101694. PubMed  Europe PubMed DOI  PMC  EPMC
  27. Zanini,G., Selleri,V., De Gaetano,A., Gibellini,L., Malerba,M., Mattioli,A.V., Nasi,M., Apostolova,N. and Pinti,M.<br>Differential Expression of Lonp1 Isoforms in Cancer Cells<br>Cells (2022) 11 PubMed  Europe PubMed DOI  PMC  EPMC
    28. 2021
  28. Ferezin,C.C., Basei,F.L., Melo-Hanchuk,T.D., de Oliveira,A.L., Peres de Oliveira,A., Mori,M.P., de Souza-Pinto,N.C. and Kobarg,J.<br>NEK5 interacts with LonP1 and its kinase activity is essential for the regulation of mitochondrial functions and mtDNA maintenance<br>FEBS Open Bio (2021) 11, 546-563. PubMed  Europe PubMed DOI
  29. Kim,J.E., Park,H., Kim,T.H. and Kang,T.C.<br>LONP1 Regulates Mitochondrial Accumulations of HMGB1 and Caspase-3 in CA1 and PV Neurons Following Status Epilepticus<br>Int J Mol Sci (2021) 22 PubMed  Europe PubMed DOI  PMC  EPMC  K
  30. Kingsley,L.J., He,X., McNeill,M., Nelson,J., Nikulin,V., Ma,Z., Lu,W., Zhou,V.W., Manuia,M., Kreusch,A., Gao,M.Y., Witmer,D., Vaillancourt,M.T., Lu,M., Greenblatt,S., Lee,C., Vashisht,A., Bender,S., Spraggon,G., Michellys,P.Y., Jia,Y., Haling,J.R. and Lelais,G.<br>Structure-Based Design of Selective LONP1 Inhibitors for Probing In Vitro Biology<br>J Med Chem (2021) 64, 4857-4869. PubMed  Europe PubMed DOI  I
  31. Maneix,L., Sweeney,M.A., Lee,S., Iakova,P., Moree,S.E., Sahin,E., Lulla,P., Yellapragada,S.V., Tsai,F.T.F. and Catic,A.<br>The Mitochondrial Protease LonP1 Promotes Proteasome Inhibitor Resistance in Multiple Myeloma<br>Cancers (Basel) (2021) 13 PubMed  Europe PubMed DOI  PMC  EPMC
  32. Matsushima,Y., Takahashi,K., Yue,S., Fujiyoshi,Y., Yoshioka,H., Aihara,M., Setoyama,D., Uchiumi,T., Fukuchi,S. and Kang,D.<br>Mitochondrial Lon protease is a gatekeeper for proteins newly imported into the matrix<br>Commun Biol (2021) 4, 974-974. PubMed  Europe PubMed DOI  PMC  EPMC
  33. Pollecker,K., Sylvester,M. and Voos,W.<br>Proteomic analysis demonstrates the role of the quality control protease LONP1 in mitochondrial protein aggregation<br>J Biol Chem (2021) , 101134-101134. PubMed  Europe PubMed DOI  K
  34. Sanchez-Lanzas,R. and Castano,J.G.<br>Mitochondrial LonP1 protease is implicated in the degradation of unstable Parkinson's disease-associated DJ-1/PARK 7 missense mutants<br>Sci Rep (2021) 11, 7320-7320. PubMed  Europe PubMed DOI  PMC  EPMC
  35. Shin,C.S., Meng,S., Garbis,S.D., Moradian,A., Taylor,R.W., Sweredoski,M.J., Lomenick,B. and Chan,D.C.<br>LONP1 and mtHSP70 cooperate to promote mitochondrial protein folding<br>Nat Commun (2021) 12, 265-265. PubMed  Europe PubMed DOI  PMC  EPMC
  36. Shin,M., Watson,E.R., Song,A.S., Mindrebo,J.T., Novick,S.J., Griffin,P.R., Wiseman,R.L. and Lander,G.C.<br>Structures of the human LONP1 protease reveal regulatory steps involved in protease activation<br>Nat Commun (2021) 12, 3239-3239. PubMed  Europe PubMed DOI  PMC  EPMC
    37. 2020
  37. Besse,A., Brezavar,D., Hanson,J., Larson,A. and Bonnen,P.E.<br>LONP1 de novo dominant mutation causes mitochondrial encephalopathy with loss of LONP1 chaperone activity and excessive LONP1 proteolytic activity<br>Mitochondrion (2020) 51, 68-78. PubMed  Europe PubMed DOI  M
  38. Li,J., Agarwal,E., Bertolini,I., Seo,J.H., Caino,M.C., Ghosh,J.C., Kossenkov,A.V., Liu,Q., Tang,H.Y., Goldman,A.R., Languino,L.R., Speicher,D.W. and Altieri,D.C.<br>The mitophagy effector FUNDC1 controls mitochondrial reprogramming and cellular plasticity in cancer cells<br>Sci Signal (2020) 13 PubMed  Europe PubMed DOI
  39. Matange,N.<br>Highly contingent phenotypes of Lon protease deficiency in Escherichia coli upon antibiotic challenge<br>J Bacteriol (2020) 202, e00561-19-e00561-19. PubMed  Europe PubMed DOI
  40. Sha,Z., Fishovitz,J., Wang,S., Chilakala,S., Xu,Y. and Lee,I.<br>A selective fluorogenic peptide substrate for the human mitochondrial ATP-dependent protease complex ClpXP<br>Chembiochem (2020) 21, 2037-2048. PubMed  Europe PubMed DOI  A  P  I
  41. Venkatesh,S. and Suzuki,C.K.<br>Cell stress management by the mitochondrial LonP1 protease - insights into mitigating developmental, oncogenic and cardiac stress<br>Mitochondrion (2020) 51, 46-61. PubMed  Europe PubMed DOI
  42. Voos,W. and Pollecker,K.<br>The mitochondrial Lon protease: novel functions off the beaten track?<br>Biomolecules (2020) 10 PubMed  Europe PubMed DOI
    43. 2019
  43. Ghosh,J.C., Seo,J.H., Agarwal,E., Wang,Y., Kossenkov,A.V., Tang,H.Y., Speicher,D.W. and Altieri,D.C.<br>Akt phosphorylation of mitochondrial Lonp1 protease enables oxidative metabolism and advanced tumor traits<br>Oncogene (2019) 38, 6926-6939. PubMed  Europe PubMed DOI
  44. Nimmo,G.A.M., Venkatesh,S., Pandey,A.K., Marshall,C.R., Hazrati,L.N., Blaser,S., Ahmed,S., Cameron,J., Singh,K., Ray,P.N., Suzuki,C.K. and Yoon,G.<br>Bi-allelic mutations of LONP1 encoding the mitochondrial LonP1 protease cause pyruvate dehydrogenase deficiency and profound neurodegeneration with progressive cerebellar atrophy<br>Hum Mol Genet (2019) 28, 290-306. PubMed  Europe PubMed DOI  M
  45. Pandey,A.K. and Venkatesh,S.<br>Protein quality control at the interface of endoplasmic reticulum and mitochondria by Lon protease<br>Br J Pharmacol (2019) 176, 505-507. PubMed  Europe PubMed DOI
  46. Venkatesh,S., Li,M., Saito,T., Tong,M., Rashed,E., Mareedu,S., Zhai,P., Barcena,C., Lopez-Otin,C., Yehia,G., Sadoshima,J. and Suzuki,C.K.<br>Mitochondrial LonP1 protects cardiomyocytes from ischemia/reperfusion injury in vivo<br>J Mol Cell Cardiol (2019) 128, 38-50. PubMed  Europe PubMed DOI
    47. 2018
  47. He,L., Luo,D., Yang,F., Li,C., Zhang,X., Deng,H. and Zhang,J.R.<br>Multiple domains of bacterial and human Lon proteases define substrate selectivity<br>Emerg Microbes Infect (2018) 7, 149-149. PubMed  Europe PubMed DOI  PMC  EPMC  P
  48. Khan,A.O. and AlBakri,A.<br>Clinical features of LONP1-related infantile cataract<br>J AAPOS (2018) 22, 229-231. PubMed  Europe PubMed DOI  M
  49. Pareek,G., Thomas,R.E., Vincow,E.S., Morris,D.R. and Pallanck,L.J.<br>Lon protease inactivation in Drosophila causes unfolded protein stress and inhibition of mitochondrial translation<br>Cell Death Discov (2018) 5, 51-51. PubMed  Europe PubMed DOI  PMC  EPMC
  50. Pareek,G. and Pallanck,L.J.<br>Inactivation of Lon protease reveals a link between mitochondrial unfolded protein stress and mitochondrial translation inhibition<br>Cell Death Dis (2018) 9, 1168-1168. PubMed  Europe PubMed DOI  PMC  EPMC  K
  51. Zurita Rendon,O. and Shoubridge,E.A.<br>LONP1 is required for maturation of a subset of mitochondrial proteins and its loss elicits an integrated stress response<br>Mol Cell Biol (2018) 38, e00412-17-e00412-17. PubMed  Europe PubMed DOI
    52. 2017
  52. Cui,X., Wei,Y., Xie,X.L., Chen,L.N. and Zhang,S.H.<br>Mitochondrial and peroxisomal Lon proteases play opposing roles in reproduction and growth but co-function in the normal development, stress resistance and longevity of Thermomyces lanuginosus<br>Fungal Genet Biol (2017) 103, 42-54. PubMed  Europe PubMed DOI
  53. Inui,T., Anzai,M., Takezawa,Y., Endo,W., Kakisaka,Y., Kikuchi,A., Onuma,A., Kure,S., Nishino,I., Ohba,C., Saitsu,H., Matsumoto,N. and Haginoya,K.<br>A novel mutation in the proteolytic domain of LONP1 causes atypical CODAS syndrome<br>J Hum Genet (2017) 62, 653-655. PubMed  Europe PubMed DOI  M
  54. Pomatto,L.C., Carney,C., Shen,B., Wong,S., Halaszynski,K., Salomon,M.P., Davies,K.J. and Tower,J.<br>The mitochondrial Lon protease is required for age-specific and sex-specific adaptation to oxidative stress<br>Curr Biol (2017) 27, 1-15. PubMed  Europe PubMed DOI
    55. 2016
  55. Bota,D.A. and Davies,K.J.<br>Mitochondrial Lon protease in human disease and aging: including an etiologic classification of Lon-related diseases and disorders<br>Free Radic Biol Med (2016) 100, 188-198. PubMed  Europe PubMed DOI
  56. Ciesielski,S.J., Schilke,B., Marszalek,J. and Craig,E.A.<br>Protection of scaffold protein Isu from degradation by the Lon protease Pim 1 as a component of Fe-S cluster biogenesis regulation<br>Mol Biol Cell (2016) 27, 1060-1068. PubMed  Europe PubMed DOI
  57. Gibellini,L., DeBiasi,S., Nasi,M., Iannone,A., Cossarizza,A. and Pinti,M.<br>Mitochondrial proteases as emerging pharmacological targets<br>Curr Pharm Des (2016) 22, 2679-2688. PubMed  Europe PubMed  T
  58. Kereiche,S., Kovacik,L., Bednar,J., Pevala,V., Kunova,N., Ondrovicova,G., Bauer,J., Ambro,L., Bellova,J., Kutejova,E. and Raska,I.<br>The N-terminal domain plays a crucial role in the structure of a full-length human mitochondrial Lon protease<br>Sci Rep (2016) 6, 33631-33631. PubMed  Europe PubMed DOI  PMC  EPMC  S
  59. Pinti,M., Gibellini,L., Nasi,M., De Biasi,S., Bortolotti,C.A., Iannone,A. and Cossarizza,A.<br>Emerging role of Lon protease as a master regulator of mitochondrial functions<br>Biochim Biophys Acta (2016) 1857, 1300-1306. PubMed  Europe PubMed DOI  M
  60. Pryde,K.R., Taanman,J.W. and Schapira,A.H.<br>A LON-ClpP proteolytic axis degrades complex I to extinguish ROS production in depolarized mitochondria<br>Cell Rep (2016) 17, 2522-2531. PubMed  Europe PubMed DOI
    61. 2015
  61. Bezawork-Geleta,A., Brodie,E.J., Dougan,D.A. and Truscott,K.N.<br>LON is the master protease that protects against protein aggregation in human mitochondria through direct degradation of misfolded proteins<br>Sci Rep (2015) 5, 17397-17397. PubMed  Europe PubMed DOI  PMC  EPMC
  62. Cui,X., Wei,Y., Wang,Y.H., Li,J., Wong,F.L., Zheng,Y.J., Yan,H., Liu,S.S., Liu,J.L., Jia,B.L. and Zhang,S.H.<br>Proteins interacting with mitochondrial ATP-dependent Lon protease (MAP1) in Magnaporthe oryzae are involved in rice blast disease<br>Mol Plant Pathol (2015) 16, 847-859. PubMed  Europe PubMed DOI
  63. Dikoglu,E., Alfaiz,A., Gorna,M., Bertola,D., Chae,J.H., Cho,T.J., Derbent,M., Alanay,Y., Guran,T., Kim,O.H., Llerenar,J.C., Jr., Yamamoto,G., Superti-Furga,G., Reymond,A., Xenarios,I., Stevenson,B., Campos-Xavier,B., Bonafe,L., Superti-Furga,A. and Unger,S.<br>Mutations in LONP1, a mitochondrial matrix protease, cause CODAS syndrome<br>Am J Med Genet A (2015) 167, 1501-1509. PubMed  Europe PubMed DOI  M
  64. Gibellini,L., Pinti,M., Bartolomeo,R., De Biasi,S., Cormio,A., Musicco,C., Carnevale,G., Pecorini,S., Nasi,M., De Pol,A. and Cossarizza,A.<br>Inhibition of Lon protease by triterpenoids alters mitochondria and is associated to cell death in human cancer cells<br>Oncotarget (2015) 6, 25466-25483. PubMed  Europe PubMed DOI  I
  65. Kao,T.Y., Chiu,Y.C., Fang,W.C., Cheng,C.W., Kuo,C.Y., Juan,H.F., Wu,S.H. and Lee,A.Y.<br>Mitochondrial Lon regulates apoptosis through the association with Hsp60-mtHsp70 complex<br>Cell Death Dis (2015) 6, e1642-e1642. PubMed  Europe PubMed DOI
  66. Kuo,C.Y., Chiu,Y.C., Lee,A.Y. and Hwang,T.L.<br>Mitochondrial Lon protease controls ROS-dependent apoptosis in cardiomyocyte under hypoxia<br>Mitochondrion (2015) 23, 7-16. PubMed  Europe PubMed DOI
  67. Pinti,M., Gibellini,L., Liu,Y., Xu,S., Lu,B. and Cossarizza,A.<br>Mitochondrial Lon protease at the crossroads of oxidative stress, ageing and cancer<br>Cell Mol Life Sci (2015) 72, 4807-4824. PubMed  Europe PubMed DOI  V
  68. Strauss,K.A., Jinks,R.N., Puffenberger,E.G., Venkatesh,S., Singh,K., Cheng,I., Mikita,N., Thilagavathi,J., Lee,J., Sarafianos,S., Benkert,A., Koehler,A., Zhu,A., Trovillion,V., McGlincy,M., Morlet,T., Deardorff,M., Innes,A.M., Prasad,C., Chudley,A.E., Lee,I.N. and Suzuki,C.K.<br>CODAS syndrome is associated with mutations of LONP1, encoding mitochondrial AAA+ Lon protease<br>Am J Hum Genet (2015) 96, 121-135. PubMed  Europe PubMed DOI  PMC  EPMC  M
    69. 2014
  69. Ambro,L., Pevala,V., Ondrovicova,G., Bellova,J., Kunova,N., Kutejova,E. and Bauer,J.<br>Mutations to a glycine loop in the catalytic site of human Lon changes its protease, peptidase and ATPase activities<br>FEBS J (2014) 281, 1784-1797. PubMed  Europe PubMed DOI
  70. Bahat,A., Perlberg,S., Melamed-Book,N., Lauria,I., Langer,T. and Orly,J.<br>StAR enhances transcription of genes encoding the mitochondrial proteases involved in its own degradation<br>Mol Endocrinol (2014) 28, 208-224. PubMed  Europe PubMed DOI
  71. Bayot,A., Gareil,M., Chavatte,L., Hamon,M.P., L'hermitte-Stead,C., Beaumatin,F., Priault,M., Rustin,P., Lombes,A., Friguet,B. and Bulteau,A.L.<br>Effect of Lon protease knockdown on mitochondrial function in HeLa cells<br>Biochimie (2014) 100, 38-47. PubMed  Europe PubMed DOI  K
  72. Gibellini,L., Pinti,M., Boraldi,F., Giorgio,V., Bernardi,P., Bartolomeo,R., Nasi,M., De Biasi,S., Missiroli,S., Carnevale,G., Losi,L., Tesei,A., Pinton,P., Quaglino,D. and Cossarizza,A.<br>Silencing of mitochondrial Lon protease deeply impairs mitochondrial proteome and function in colon cancer cells<br>FASEB J (2014) 28, 5122-5135. PubMed  Europe PubMed DOI  I
  73. Hoshino,A., Okawa,Y., Ariyoshi,M., Kaimoto,S., Uchihashi,M., Fukai,K., Iwai-Kanai,E. and Matoba,S.<br>Oxidative post-translational modifications develop LONP1 dysfunction in pressure overload heart failure<br>Circ Heart Fail (2014) 7, 500-509. PubMed  Europe PubMed DOI
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