Merops Switch to
Inhibitors

Peptidase

Family Clan SEARCHES BLAST
MEROPS SUBMISSIONS

Other
information

Literature for peptidase S16.002: PIM1 peptidase

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

(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.
    LONP-1 deficiency causes dysregulated protein synthesis within mitochondria that is restored by mitoribosomal mutations
    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.
    Lon/Pim1 mediated degradation of presequence translocase-associated motor components Pam16 and Pam18 in Saccharomyces cerevisiae
    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.
    LONP1 regulation of mitochondrial protein folding provides insight into beta cell failure in type 2 diabetes
    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.
    Identifying a novel Mecp2-mediated epigenetic mechanism controlling Lonp1 in the hippocampus and its disruption by aging
    Sci Rep (2025) 15, 40887-40887. PubMed  Europe PubMed DOI  PMC  EPMC
  5. Mindrebo,J.T. and Lander,G.C.
    Substrate-dependent activation of LONP1 informs on proteolytic regulation and ATPase motor function
    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.
    Sulforaphane downregulates mitochondrial TIGAR via inhibiting mitochondrial transmembrane assembly and LONP1/CASP3 axis causing apoptosis
    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.
    LONP1 Variants Are Associated With Clinically Diverse Phenotypes
    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.
    LONP1 Promotes Hepatocarcinogenesis by Degrading ACO2 to Alleviate Ferroptosis
    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.
    Artemisinins ameliorate polycystic ovarian syndrome by mediating LONP1-CYP11A1 interaction
    Science (2024) 384, eadk5382-eadk5382. PubMed  Europe PubMed DOI  I
  10. Shetty,R., Noland,R., Nandi,G. and Suzuki,C.K.
    Powering down the mitochondrial LonP1 protease: a novel strategy for anticancer therapeutics
    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.
    The protease Lon prolongs insect lifespan by responding to reactive oxygen species and degrading mitochondrial transcription factor A
    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.
    LONP1 alleviates ageing-related renal fibrosis by maintaining mitochondrial homeostasis
    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.
    Discovery and Validation of Novel LonP1 and Proteasome Inhibitor in IDH1-R132H Malignant Astrocytoma Models
    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.
    Proteolytic rewiring of mitochondria by LONP1 directs cell identity switching of adipocytes
    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.
    LonP1 Links Mitochondria-ER Interaction to Regulate Heart Function
    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.
    Differential sensitivity of the yeast Lon protease Pim1p to impaired mitochondrial respiration
    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.
    Inhibition of Lonp1 induces mitochondrial remodeling and autophagy suppression in cervical cancer cells
    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.
    Down-regulation of Lon protease 1 lysine crotonylation aggravates mitochondrial dysfunction in polycystic ovary syndrome
    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.
    The Role of Lonp1 on Mitochondrial Functions during Cardiovascular and Muscular Diseases
    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.
    Evidence for mitochondrial Lonp1 expression in the nucleus
    Sci Rep (2022) 12, 10877-10877. PubMed  Europe PubMed DOI  PMC  EPMC
  21. Hackett,P.T., Jia,X., Li,L. and Ward,D.M.
    Posttranslational regulation of mitochondrial frataxin and identification of compounds that increase frataxin levels in Friedreich's ataxia
    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.
    LONP1 downregulation with ageing contributes to osteoarthritis via mitochondrial dysfunction
    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.
    Catalytic cycling of human mitochondrial Lon protease
    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.
    The mitochondrial protease LONP1 maintains oocyte development and survival by suppressing nuclear translocation of AIFM1 in mammals
    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.
    Organismal and Cellular Stress Responses upon Disruption of Mitochondrial Lonp1 Protease
    Cells (2022) 11 PubMed  Europe PubMed DOI  PMC  EPMC  K  I
  26. Yang,J., Song,A.S., Wiseman,R.L. and Lander,G.C.
    Cryo-EM structure of hexameric yeast Lon protease (PIM1) highlights the importance of conserved structural elements
    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.
    Differential Expression of Lonp1 Isoforms in Cancer Cells
    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.
    NEK5 interacts with LonP1 and its kinase activity is essential for the regulation of mitochondrial functions and mtDNA maintenance
    FEBS Open Bio (2021) 11, 546-563. PubMed  Europe PubMed DOI
  29. Kim,J.E., Park,H., Kim,T.H. and Kang,T.C.
    LONP1 Regulates Mitochondrial Accumulations of HMGB1 and Caspase-3 in CA1 and PV Neurons Following Status Epilepticus
    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.
    Structure-Based Design of Selective LONP1 Inhibitors for Probing In Vitro Biology
    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.
    The Mitochondrial Protease LonP1 Promotes Proteasome Inhibitor Resistance in Multiple Myeloma
    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.
    Mitochondrial Lon protease is a gatekeeper for proteins newly imported into the matrix
    Commun Biol (2021) 4, 974-974. PubMed  Europe PubMed DOI  PMC  EPMC
  33. Pollecker,K., Sylvester,M. and Voos,W.
    Proteomic analysis demonstrates the role of the quality control protease LONP1 in mitochondrial protein aggregation
    J Biol Chem (2021) , 101134-101134. PubMed  Europe PubMed DOI  K
  34. Sanchez-Lanzas,R. and Castano,J.G.
    Mitochondrial LonP1 protease is implicated in the degradation of unstable Parkinson's disease-associated DJ-1/PARK 7 missense mutants
    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.
    LONP1 and mtHSP70 cooperate to promote mitochondrial protein folding
    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.
    Structures of the human LONP1 protease reveal regulatory steps involved in protease activation
    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.
    LONP1 de novo dominant mutation causes mitochondrial encephalopathy with loss of LONP1 chaperone activity and excessive LONP1 proteolytic activity
    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.
    The mitophagy effector FUNDC1 controls mitochondrial reprogramming and cellular plasticity in cancer cells
    Sci Signal (2020) 13 PubMed  Europe PubMed DOI
  39. Matange,N.
    Highly contingent phenotypes of Lon protease deficiency in Escherichia coli upon antibiotic challenge
    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.
    A selective fluorogenic peptide substrate for the human mitochondrial ATP-dependent protease complex ClpXP
    Chembiochem (2020) 21, 2037-2048. PubMed  Europe PubMed DOI  A  P  I
  41. Venkatesh,S. and Suzuki,C.K.
    Cell stress management by the mitochondrial LonP1 protease - insights into mitigating developmental, oncogenic and cardiac stress
    Mitochondrion (2020) 51, 46-61. PubMed  Europe PubMed DOI
  42. Voos,W. and Pollecker,K.
    The mitochondrial Lon protease: novel functions off the beaten track?
    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.
    Akt phosphorylation of mitochondrial Lonp1 protease enables oxidative metabolism and advanced tumor traits
    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.
    Bi-allelic mutations of LONP1 encoding the mitochondrial LonP1 protease cause pyruvate dehydrogenase deficiency and profound neurodegeneration with progressive cerebellar atrophy
    Hum Mol Genet (2019) 28, 290-306. PubMed  Europe PubMed DOI  M
  45. Pandey,A.K. and Venkatesh,S.
    Protein quality control at the interface of endoplasmic reticulum and mitochondria by Lon protease
    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.
    Mitochondrial LonP1 protects cardiomyocytes from ischemia/reperfusion injury in vivo
    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.
    Multiple domains of bacterial and human Lon proteases define substrate selectivity
    Emerg Microbes Infect (2018) 7, 149-149. PubMed  Europe PubMed DOI  PMC  EPMC  P
  48. Khan,A.O. and AlBakri,A.
    Clinical features of LONP1-related infantile cataract
    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.
    Lon protease inactivation in Drosophila causes unfolded protein stress and inhibition of mitochondrial translation
    Cell Death Discov (2018) 5, 51-51. PubMed  Europe PubMed DOI  PMC  EPMC
  50. Pareek,G. and Pallanck,L.J.
    Inactivation of Lon protease reveals a link between mitochondrial unfolded protein stress and mitochondrial translation inhibition
    Cell Death Dis (2018) 9, 1168-1168. PubMed  Europe PubMed DOI  PMC  EPMC  K
  51. Zurita Rendon,O. and Shoubridge,E.A.
    LONP1 is required for maturation of a subset of mitochondrial proteins and its loss elicits an integrated stress response
    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.
    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
    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.
    A novel mutation in the proteolytic domain of LONP1 causes atypical CODAS syndrome
    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.
    The mitochondrial Lon protease is required for age-specific and sex-specific adaptation to oxidative stress
    Curr Biol (2017) 27, 1-15. PubMed  Europe PubMed DOI
    55. 2016
  55. Bota,D.A. and Davies,K.J.
    Mitochondrial Lon protease in human disease and aging: including an etiologic classification of Lon-related diseases and disorders
    Free Radic Biol Med (2016) 100, 188-198. PubMed  Europe PubMed DOI
  56. Ciesielski,S.J., Schilke,B., Marszalek,J. and Craig,E.A.
    Protection of scaffold protein Isu from degradation by the Lon protease Pim 1 as a component of Fe-S cluster biogenesis regulation
    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.
    Mitochondrial proteases as emerging pharmacological targets
    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.
    The N-terminal domain plays a crucial role in the structure of a full-length human mitochondrial Lon protease
    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.
    Emerging role of Lon protease as a master regulator of mitochondrial functions
    Biochim Biophys Acta (2016) 1857, 1300-1306. PubMed  Europe PubMed DOI  M
  60. Pryde,K.R., Taanman,J.W. and Schapira,A.H.
    A LON-ClpP proteolytic axis degrades complex I to extinguish ROS production in depolarized mitochondria
    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.
    LON is the master protease that protects against protein aggregation in human mitochondria through direct degradation of misfolded proteins
    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.
    Proteins interacting with mitochondrial ATP-dependent Lon protease (MAP1) in Magnaporthe oryzae are involved in rice blast disease
    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.
    Mutations in LONP1, a mitochondrial matrix protease, cause CODAS syndrome
    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.
    Inhibition of Lon protease by triterpenoids alters mitochondria and is associated to cell death in human cancer cells
    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.
    Mitochondrial Lon regulates apoptosis through the association with Hsp60-mtHsp70 complex
    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.
    Mitochondrial Lon protease controls ROS-dependent apoptosis in cardiomyocyte under hypoxia
    Mitochondrion (2015) 23, 7-16. PubMed  Europe PubMed DOI
  67. Pinti,M., Gibellini,L., Liu,Y., Xu,S., Lu,B. and Cossarizza,A.
    Mitochondrial Lon protease at the crossroads of oxidative stress, ageing and cancer
    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.
    CODAS syndrome is associated with mutations of LONP1, encoding mitochondrial AAA+ Lon protease
    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.
    Mutations to a glycine loop in the catalytic site of human Lon changes its protease, peptidase and ATPase activities
    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.
    StAR enhances transcription of genes encoding the mitochondrial proteases involved in its own degradation
    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.
    Effect of Lon protease knockdown on mitochondrial function in HeLa cells
    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.
    Silencing of mitochondrial Lon protease deeply impairs mitochondrial proteome and function in colon cancer cells
    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.
    Oxidative post-translational modifications develop LONP1 dysfunction in pressure overload heart failure
    Circ Heart Fail (2014) 7, 500-509. PubMed  Europe PubMed DOI
  74. Thomas,R.E., Andrews,L.A., Burman,J.L., Lin,W.Y. and Pallanck,L.J.
    PINK1-Parkin pathway activity is regulated by degradation of PINK1 in the mitochondrial matrix
    PLoS Genet (2014) 10, e1004279-e1004279. PubMed  Europe PubMed DOI  PMC  EPMC
  75. Zhang,B., Shen,X.L., Liang,R., Li,Y., Huang,K., Zhao,C., Luo,Y. and Xu,W.
    Protective role of the mitochondrial Lon protease 1 in ochratoxin A-induced cytotoxicity in HEK293 cells
    J Proteomics (2014) 101, 154-168. PubMed  Europe PubMed DOI
    76. 2013
  76. Teng,H., Wu,B., Zhao,K., Yang,G., Wu,L. and Wang,R.
    Oxygen-sensitive mitochondrial accumulation of cystathionine beta-synthase mediated by Lon protease
    Proc Natl Acad Sci U S A (2013) 110, 12679-12684. PubMed  Europe PubMed DOI
    77. 2012
  77. Bernstein,S.H., Venkatesh,S., Li,M., Lee,J., Lu,B., Hilchey,S.P., Morse,K.M., Metcalfe,H.M., Skalska,J., Andreeff,M., Brookes,P.S. and Suzuki,C.K.
    The mitochondrial ATP-dependent Lon protease: a novel target in lymphoma death mediated by the synthetic triterpenoid CDDO and its derivatives
    Blood (2012) 119, 3321-3329. PubMed  Europe PubMed DOI  PMC  EPMC  I
  78. Lee,J., Sundararajan,V., Lee,I. and Suzuki,C.K.
    Mitochondrial ATP-dependent Lon protease
    [ISSN:978-0-12-407742-3] (2012) 3, 3533-3540. DOI
  79. Matsushima,Y. and Kaguni,L.S.
    Matrix proteases in mitochondrial DNA function
    Biochim Biophys Acta (2012) 1819, 1080-1087. PubMed  Europe PubMed DOI
    80. 2011
  80. Fishovitz,J., Li,M., Frase,H., Hudak,J., Craig,S., Ko,K., Berdis,A.J., Suzuki,C.K. and Lee,I.
    Active-site-directed chemical tools for profiling mitochondrial lon protease
    ACS Chem Biol (2011) 6, 781-788. PubMed  Europe PubMed DOI  P
  81. Kindrachuk,K.N., Fernandez,L., Bains,M. and Hancock,R.E.
    Involvement of an ATP-dependent protease, PA0779/AsrA, in inducing heat shock in response to tobramycin in Pseudomonas aeruginosa
    Antimicrob Agents Chemother (2011) 55, 1874-1882. PubMed  Europe PubMed DOI
  82. Lee,H.J., Chung,K., Lee,H., Lee,K., Lim,J.H. and Song,J.
    Downregulation of mitochondrial lon protease impairs mitochondrial function and causes hepatic insulin resistance in human liver SK-HEP-1 cells
    Diabetologia (2011) 54, 1437-1446. PubMed  Europe PubMed DOI  K
  83. Pinti,M., Gibellini,L., De Biasi,S., Nasi,M., Roat,E., O'Connor,J.E. and Cossarizza,A.
    Functional characterization of the promoter of the human Lon protease gene
    Mitochondrion (2011) 11, 200-206. PubMed  Europe PubMed DOI
  84. Tian,Q., Li,T., Hou,W., Zheng,J., Schrum,L.W. and Bonkovsky,H.L.
    Lon peptidase 1 (LONP1)-dependent breakdown of mitochondrial 5-aminolevulinic acid synthase protein by heme in human liver cells
    J Biol Chem (2011) 286, 26424-26430. PubMed  Europe PubMed DOI
    85. 2010
  85. Garcia-Nafria,J., Ondrovicova,G., Blagova,E., Levdikov,V.M., Bauer,J.A., Suzuki,C.K., Kutejova,E., Wilkinson,A.J. and Wilson,K.S.
    Structure of the catalytic domain of the human mitochondrial Lon protease: proposed relation of oligomer formation and activity
    Protein Sci (2010) 19, 987-999. PubMed  Europe PubMed DOI  S
  86. Matsushima,Y., Goto,Y. and Kaguni,L.S.
    Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM)
    Proc Natl Acad Sci U S A (2010) 107, 18410-18415. PubMed  Europe PubMed DOI
  87. Pinti,M., Gibellini,L., Guaraldi,G., Orlando,G., Gant,T.W., Morselli,E., Nasi,M., Salomoni,P., Mussini,C. and Cossarizza,A.
    Upregulation of nuclear-encoded mitochondrial LON protease in HAART-treated HIV-positive patients with lipodystrophy: implications for the pathogenesis of the disease
    AIDS (2010) 24, 841-850. PubMed  Europe PubMed DOI
    88. 2009
  88. Coleman,J.L., Katona,L.I., Kuhlow,C., Toledo,A., Okan,N.A., Tokarz,R. and Benach,J.L.
    Evidence that two ATP-dependent (Lon) proteases in Borrelia burgdorferi serve different functions
    PLoS Pathog (2009) 5, e1000676-e1000676. PubMed  Europe PubMed DOI  PMC  EPMC
  89. Ngo,J.K. and Davies,K.J.
    Mitochondrial Lon protease is a human stress protein
    Free Radic Biol Med (2009) 46, 1042-1048. PubMed  Europe PubMed DOI
  90. Rigas,S., Daras,G., Sweetlove,L.J. and Hatzopoulos,P.
    Mitochondria biogenesis via Lon1 selective proteolysis: who dares to live for ever?
    Plant Signal Behav (2009) 4, 221-224. PubMed  Europe PubMed  PMC  EPMC
    91. 2008
  91. Bayot,A., Basse,N., Lee,I., Gareil,M., Pirotte,B., Bulteau,A.L., Friguet,B. and Reboud-Ravaux,M.
    Towards the control of intracellular protein turnover: mitochondrial Lon protease inhibitors versus proteasome inhibitors
    Biochimie (2008) 90, 260-269. PubMed  Europe PubMed DOI  I
  92. Chen,S.H., Suzuki,C.K. and Wu,S.H.
    Thermodynamic characterization of specific interactions between the human Lon protease and G-quartet DNA
    Nucleic Acids Res (2008) 36, 1273-1287. PubMed  Europe PubMed DOI  PMC  EPMC
  93. Lee,I. and Suzuki,C.K.
    Functional mechanics of the ATP-dependent Lon protease - lessons from endogenous protein and synthetic peptide substrates
    Biochim Biophys Acta (2008) 1784, 727-735. PubMed  Europe PubMed DOI  PMC  EPMC  V
  94. Stanyer,L., Jorgensen,W., Hori,O., Clark,J.B. and Heales,S.J.
    Inactivation of brain mitochondrial Lon protease by peroxynitrite precedes electron transport chain dysfunction
    Neurochem Int (2008) 53, 95-101. PubMed  Europe PubMed DOI
    95. 2007
  95. Lu,B., Yadav,S., Shah,P.G., Liu,T., Tian,B., Pukszta,S., Villaluna,N., Kutejova,E., Newlon,C.S., Santos,J.H. and Suzuki,C.K.
    Roles for the human ATP-dependent Lon protease in mitochondrial DNA maintenance
    J Biol Chem (2007) 282, 17363-17374. PubMed  Europe PubMed DOI
  96. Ngo,J.K. and Davies,K.J.
    Importance of the lon protease in mitochondrial maintenance and the significance of declining lon in aging
    Ann N Y Acad Sci (2007) 1119, 78-87. PubMed  Europe PubMed DOI  V
  97. Ostersetzer,O., Kato,Y., Adam,Z. and Sakamoto,W.
    Multiple intracellular locations of Lon protease in Arabidopsis: evidence for the localization of AtLon4 to chloroplasts
    Plant Cell Physiol (2007) 48, 881-885. PubMed  Europe PubMed DOI
    98. 2005
  98. [YEAR:1-3-2005]Bota,D.A., Ngo,J.K. and Davies,K.J.
    Downregulation of the human Lon protease impairs mitochondrial structure and function and causes cell death
    Free Radic Biol Med (1-3-2005) 38, 665-677. PubMed  Europe PubMed DOI
  99. [YEAR:1-6-2005]Levine,R.L.
    Commentary on "Downregulation of the human Lon protease impairs mitochondrial structure and function and causes cell death" by D.A. Bota, J.K. Ngo, and K.J.A. Davies
    Free Radic Biol Med (1-6-2005) 38, 1445-1446. PubMed  Europe PubMed DOI
  100. von Janowsky,B., Knapp,K., Major,T., Krayl,M., Guiard,B. and Voos,W.
    Structural properties of substrate proteins determine their proteolysis by the mitochondrial AAA+ protease Pim1
    Biol Chem (2005) 386, 1307-1317. PubMed  Europe PubMed DOI
    101. 2004
  101. Langer,T.
    PIM1 mitochondrial endopeptidase
    [ISSN:0-12-079610-4] (2004) 2, 2003-2006.  V
  102. [YEAR:22-1-2004]Liu,T., Lu,B., Lee,I., Ondrovicova,G., Kutejova,E. and Suzuki,C.K.
    DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate
    J Biol Chem (22-1-2004) 279, 13902-13910. PubMed  Europe PubMed DOI
    103. 2003
  103. [YEAR:13-3-2003]Lu,B., Liu,T., Crosby,J.A., Thomas-Wohlever,J., Lee,I. and Suzuki,C.K.
    The ATP-dependent Lon protease of Mus musculus is a DNA-binding protein that is functionally conserved between yeast and mammals
    Gene (13-3-2003) 306, 45-55. PubMed  Europe PubMed DOI
    104. 2002
  104. Bota,D.A. and Davies,K.J.
    Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism
    Nat Cell Biol (2002) 4, 674-680. PubMed  Europe PubMed DOI
  105. [YEAR:15-10-2002]Zhu,Y., Wang,M., Lin,H., Huang,C., Shi,X. and Luo,J.
    Epidermal growth factor up-regulates the transcription of mouse Lon homology ATP-dependent protease through extracellular signal-regulated protein kinase- and phosphatidylinositol-3-kinase-dependent pathways
    Exp Cell Res (15-10-2002) 280, 97-106. PubMed  Europe PubMed DOI
    106. 2001
  106. Adam,Z., Adamska,I., Nakabayashi,K., Ostersetzer,O., Haussuhl,K., Manuell,A., Zheng,B., Vallon,O., Rodermel,S.R., Shinozaki,K. and Clarke,A.K.
    Chloroplast and mitochondrial proteases in Arabidopsis. A proposed nomenclature
    Plant Physiol (2001) 125, 1912-1918. PubMed  Europe PubMed DOI  PMC  EPMC
  107. [YEAR:1-4-2001]Lee,I. and Berdis,A.J.
    Adenosine triphosphate-dependent degradation of a fluorescent lambda N substrate mimic by lon protease
    Anal Biochem (1-4-2001) 291, 74-83. PubMed  Europe PubMed DOI
    108. 2000
  108. [YEAR:4-1-2000]Birghan,C., Mundt,E. and Gorbalenya,A.E.
    A non-canonical Lon proteinase lacking the ATPase domain employs the Ser-Lys catalytic dyad to exercise broad control over the life cycle of a double-stranded RNA virus
    EMBO J (4-1-2000) 19, 114-123. PubMed  Europe PubMed DOI  PMC  EPMC
    109. 1998
  109. [YEAR:17-2-1998]Fu,G.K. and Markovitz,D.M.
    The human LON protease binds to mitochondrial promoters in a single-stranded, site-specific, strand-specific manner
    Biochemistry (17-2-1998) 37, 1905-1909. PubMed  Europe PubMed DOI
    110. 1997
  110. Suzuki,C.K., Rep,M., van Dijl,J.M., Suda,K., Grivell,L.A. and Schatz,G.
    ATP-dependent proteases that also chaperone protein biogenesis
    Trends Biochem Sci (1997) 22, 118-123. PubMed  Europe PubMed DOI  V
  111. [YEAR:15-12-1997]Wagner,I., Van Dyck,L., Savel'ev,A.S., Neupert,W. and Langer,T.
    Autocatalytic processing of the ATP-dependent PIM1 protease: crucial function of a pro-region for sorting to mitochondria
    EMBO J (15-12-1997) 16, 7317-7325. PubMed  Europe PubMed DOI  PMC  EPMC
    112. 1996
  112. Langer,T. and Neupert,W.
    Regulated protein degradation in mitochondria
    Experientia (1996) 52, 1069-1076. PubMed  Europe PubMed DOI
    113. 1994
  113. Amerik,A.Y., Petukhova,G.V., Grigorenko,V.G., Lykov,I.P., Yarovoi,S.V., Lipkin,V.M. and Gorbalenya,A.E.
    Cloning and sequence analysis of cDNA for a human homolog of eubacterial ATP-dependent Lon proteases
    FEBS Lett (1994) 340, 25-28. PubMed  Europe PubMed DOI
  114. Kuzela,S. and Goldberg,A.L.
    Mitochondrial ATP-dependent protease from rat liver and yeast
    Methods Enzymol (1994) 244, 376-383. PubMed  Europe PubMed DOI  V
  115. Wagner,I., Arlt,H., Van Dyck,L., Langer,T. and Neupert,W.
    Molecular chaperones cooperate with PIM1 protease in the degradation of misfolded proteins in mitochondria
    EMBO J (1994) 13, 5135-5145. PubMed  Europe PubMed  PMC  EPMC
  116. Wang,N., Maurizi,M.R., Emmert-Buck,L. and Gottesman,M.M.
    Synthesis, processing, and localization of human Lon protease
    J Biol Chem (1994) 269, 29308-29313. PubMed  Europe PubMed
  117. Watabe,S., Kohno,H., Kouyama,H., Hiroi,T., Yago,N. and Nakazawa,T.
    Purification and characterization of a substrate protein for mitochondrial ATP-dependent protease in bovine adrenal cortex
    J Biochem (1994) 115, 648-654. PubMed  Europe PubMed
    118. 1993
  118. [YEAR:1-12-1993]Wang,N., Gottesman,S., Willingham,M.C., Gottesman,M.M. and Maurizi,M.R.
    A human mitochondrial ATP-dependent protease that is highly homologous to bacterial Lon protease
    Proc Natl Acad Sci U S A (1-12-1993) 90, 11247-11251. PubMed  Europe PubMed DOI  PMC  EPMC
    119. 1985
  119. [YEAR:25-11-1985]Watabe,S. and Kimura,T.
    Adrenal cortex mitochondrial enzyme with ATP-dependent protease and protein-dependent ATPase activities. Purification and properties
    J Biol Chem (25-11-1985) 260, 14498-14504. PubMed  Europe PubMed
  120. Watabe,S. and Kimura,T.
    ATP-dependent protease in bovine adrenal cortex. Tissue specificity, subcellular localization, and partial characterization
    J Biol Chem (1985) 260, 5511-5517. PubMed  Europe PubMed
    121. 1984
  121. Charette,M.F., Henderson,G.W., Doane,L.L. and Markovitz,A.
    DNA-stimulated ATPase activity on the lon (CapR) protein
    J Bacteriol (1984) 158, 195-201. PubMed  Europe PubMed  PMC  EPMC
    122. 1982
  122. Desautels,M. and Goldberg,A.L.
    Demonstration of an ATP-dependent, vanadate-sensitive endoprotease in the matrix of rat liver mitochondria
    J Biol Chem (1982) 257, 11673-11679. PubMed  Europe PubMed
    123. 1964
  123. Adler,H.I. and Hardigree,A.A.
    Analysis of a gene controlling cell division and sensitivity to radiation in Escherichia coli
    J Bacteriol (1964) 87, 720-726. PubMed  Europe PubMed  PMC  EPMC
  124. Howard-Flanders,P., Simson,E. and Theriot,L.
    A locus that controls filament formation and sensitivity to radiation in Escherichia coli K-12
    Genetics (1964) 49, 237-246. PubMed  Europe PubMed  PMC  EPMC

feedback
Page created 8-September-2023

EMBL-EBI logo

© 2026 EBI
Terms of use