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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.)

    2025
  1. 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 Commun760, 151689-151689. PubMed  Europe PubMed DOI  K
    2. 2024
  2. 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
    Science384, eadk5382-eadk5382. PubMed  Europe PubMed DOI  I
  3. 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 Targets28, 9-15. PubMed  Europe PubMed DOI  V
  4. 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 Res1871, 119648-119648. PubMed  Europe PubMed DOI
  5. 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 Med28, e70090-e70090. PubMed  Europe PubMed DOI
    6. 2023
  6. 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 PubMed  Europe PubMed DOI  I
  7. 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 Biol25, 848-864. PubMed  Europe PubMed DOI
  8. 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)6, 0175-0175. PubMed  Europe PubMed DOI
  9. 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 Chem299, 104937-104937. PubMed  Europe PubMed DOI
  10. 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 Histochem125, 151986-151986. PubMed  Europe PubMed DOI  K
  11. 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)4, e396-e396. PubMed  Europe PubMed DOI
  12. 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)12, PubMed  Europe PubMed DOI
    13. 2022
  13. 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 Rep12, 10877-10877. PubMed  Europe PubMed DOI
  14. 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 Chem298, 101982-101982. PubMed  Europe PubMed DOI
  15. 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 Med191, 176-190. PubMed  Europe PubMed DOI  K  I
  16. Mohammed,I., Schmitz,K.A., Schenck,N., Balasopoulos,D., Topitsch,A., Maier,T. and Abrahams,J.P.
    Catalytic cycling of human mitochondrial Lon protease
    Structure30, 1254-1268. PubMed  Europe PubMed DOI
  17. 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
    EBioMedicine75, 103790-103790. PubMed  Europe PubMed DOI
  18. 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
    Cells11, PubMed  Europe PubMed DOI  K  I
  19. 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 Chem298, 101694-101694. PubMed  Europe PubMed DOI
  20. 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
    Cells11, PubMed  Europe PubMed DOI
    21. 2021
  21. 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 Bio11, 546-563. PubMed  Europe PubMed DOI
  22. 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 Sci22, PubMed  Europe PubMed DOI  K
  23. 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 Chem64, 4857-4869. PubMed  Europe PubMed DOI  I
  24. 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)13, PubMed  Europe PubMed DOI
  25. 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 Biol4, 974-974. PubMed  Europe PubMed DOI
  26. Pollecker,K., Sylvester,M. and Voos,W.
    Proteomic analysis demonstrates the role of the quality control protease LONP1 in mitochondrial protein aggregation
    J Biol Chem101134-101134. PubMed  Europe PubMed DOI  K
  27. 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 Rep11, 7320-7320. PubMed  Europe PubMed DOI
  28. 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 Commun12, 265-265. PubMed  Europe PubMed DOI
  29. 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 Commun12, 3239-3239. PubMed  Europe PubMed DOI
    30. 2020
  30. 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
    Mitochondrion51, 68-78. PubMed  Europe PubMed DOI  M
  31. Matange,N.
    Highly contingent phenotypes of Lon protease deficiency in Escherichia coli upon antibiotic challenge
    J Bacteriol202, e00561-19-e00561-19. PubMed  Europe PubMed DOI
  32. 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
    Chembiochem21, 2037-2048. PubMed  Europe PubMed DOI  A  P  I
  33. Venkatesh,S. and Suzuki,C.K.
    Cell stress management by the mitochondrial LonP1 protease - insights into mitigating developmental, oncogenic and cardiac stress
    Mitochondrion51, 46-61. PubMed  Europe PubMed DOI
  34. Voos,W. and Pollecker,K.
    The mitochondrial Lon protease: novel functions off the beaten track?
    Biomolecules10, PubMed  Europe PubMed DOI
    35. 2019
  35. 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
    Oncogene38, 6926-6939. PubMed  Europe PubMed DOI
  36. 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 Genet28, 290-306. PubMed  Europe PubMed DOI  M
  37. Pandey,A.K. and Venkatesh,S.
    Protein quality control at the interface of endoplasmic reticulum and mitochondria by Lon protease
    Br J Pharmacol176, 505-507. PubMed  Europe PubMed DOI
  38. 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 Cardiol128, 38-50. PubMed  Europe PubMed DOI
    39. 2018
  39. 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 Infect7, 149-149. PubMed  Europe PubMed DOI  P
  40. Khan,A.O. and AlBakri,A.
    Clinical features of LONP1-related infantile cataract
    J AAPOS22, 229-231. PubMed  Europe PubMed DOI  M
  41. 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 Discov5, 51-51. PubMed  Europe PubMed DOI
  42. Pareek,G. and Pallanck,L.J.
    Inactivation of Lon protease reveals a link between mitochondrial unfolded protein stress and mitochondrial translation inhibition
    Cell Death Dis9, 1168-1168. PubMed  Europe PubMed DOI  K
  43. 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 Biol38, e00412-17-e00412-17. PubMed  Europe PubMed DOI
    44. 2017
  44. 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 Biol103, 42-54. PubMed  Europe PubMed DOI
  45. 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 Genet62, 653-655. PubMed  Europe PubMed DOI  M
  46. 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 Biol27, 1-15. PubMed  Europe PubMed DOI
    47. 2016
  47. 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 Med100, 188-198. PubMed  Europe PubMed DOI
  48. 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 Cell27, 1060-1068. PubMed  Europe PubMed DOI
  49. Gibellini,L., DeBiasi,S., Nasi,M., Iannone,A., Cossarizza,A. and Pinti,M.
    Mitochondrial proteases as emerging pharmacological targets
    Curr Pharm Des22, 2679-2688. PubMed  Europe PubMed  T
  50. 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 Rep6, 33631-33631. PubMed  Europe PubMed DOI  S
  51. 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 Acta1857, 1300-1306. PubMed  Europe PubMed DOI  M
  52. 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 Rep17, 2522-2531. PubMed  Europe PubMed DOI
    53. 2015
  53. 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 Rep5, 17397-17397. PubMed  Europe PubMed DOI
  54. 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 Pathol16, 847-859. PubMed  Europe PubMed DOI
  55. 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 A167, 1501-1509. PubMed  Europe PubMed DOI  M
  56. 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
    Oncotarget6, 25466-25483. PubMed  Europe PubMed DOI  I
  57. 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 Dis6, e1642-e1642. PubMed  Europe PubMed DOI
  58. Kuo,C.Y., Chiu,Y.C., Lee,A.Y. and Hwang,T.L.
    Mitochondrial Lon protease controls ROS-dependent apoptosis in cardiomyocyte under hypoxia
    Mitochondrion23, 7-16. PubMed  Europe PubMed DOI
  59. 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 Sci72, 4807-4824. PubMed  Europe PubMed DOI  V
  60. 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 Genet96, 121-135. PubMed  Europe PubMed DOI  M
    61. 2014
  61. 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 J281, 1784-1797. PubMed  Europe PubMed DOI
  62. 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 Endocrinol28, 208-224. PubMed  Europe PubMed DOI
  63. 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
    Biochimie100, 38-47. PubMed  Europe PubMed DOI  K
  64. 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 J28, 5122-5135. PubMed  Europe PubMed DOI  I
  65. 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 Fail7, 500-509. PubMed  Europe PubMed DOI
  66. 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 Genet10, e1004279-e1004279. PubMed  Europe PubMed DOI
  67. 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 Proteomics101, 154-168. PubMed  Europe PubMed DOI
    68. 2013
  68. 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 A110, 12679-12684. PubMed  Europe PubMed DOI
    69. 2012
  69. 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
    Blood119, 3321-3329. PubMed  Europe PubMed DOI  I
  70. Lee,J., Sundararajan,V., Lee,I. and Suzuki,C.K.
    Mitochondrial ATP-dependent Lon protease
    [ISSN:978-0-12-407742-3]3, 3533-3540. DOI
  71. Matsushima,Y. and Kaguni,L.S.
    Matrix proteases in mitochondrial DNA function
    Biochim Biophys Acta1819, 1080-1087. PubMed  Europe PubMed DOI
    72. 2011
  72. 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 Biol6, 781-788. PubMed  Europe PubMed DOI  P
  73. 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 Chemother55, 1874-1882. PubMed  Europe PubMed DOI
  74. 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
    Diabetologia54, 1437-1446. PubMed  Europe PubMed DOI  K
  75. 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
    Mitochondrion11, 200-206. PubMed  Europe PubMed DOI
  76. 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 Chem286, 26424-26430. PubMed  Europe PubMed DOI
    77. 2010
  77. 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 Sci19, 987-999. PubMed  Europe PubMed DOI  S
  78. 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 A107, 18410-18415. PubMed  Europe PubMed DOI
  79. 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
    AIDS24, 841-850. PubMed  Europe PubMed DOI
    80. 2009
  80. 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 Pathog5, e1000676-e1000676. PubMed  Europe PubMed DOI
  81. Ngo,J.K. and Davies,K.J.
    Mitochondrial Lon protease is a human stress protein
    Free Radic Biol Med46, 1042-1048. PubMed  Europe PubMed DOI
  82. Rigas,S., Daras,G., Sweetlove,L.J. and Hatzopoulos,P.
    Mitochondria biogenesis via Lon1 selective proteolysis: who dares to live for ever?
    Plant Signal Behav4, 221-224. PubMed  Europe PubMed
    83. 2008
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