| 1 | ALYREF, LONP1
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| Rare and de novo variants in 827 congenital diaphragmatic hernia probands implicate LONP1 as candidate risk gene
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| Qiao L, Xu L, Yu L, Wynn J, Hernan R, Zhou X, Farkouh-Karoleski C, Krishnan US, Khlevner J, De A, Zygmunt A, Crombleholme T, Lim FY, Needelman H, Cusick RA, Mychaliska GB, Warner BW, Wagner AJ, Danko ME, Chung D, Potoka D, Kosiński P, McCulley DJ, Elfiky M, Azarow K, Fialkowski E, Schindel D, Soffer SZ, Lyon JB, Zalieckas JM, Vardarajan BN, Aspelund G, Duron VP, High FA, Sun X, Donahoe PK, Shen Y, Chung WK.
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| Am J Hum Genet. Oct 7;108(10):1964-1980. doi: 10.1016/j.ajhg.2021.08.011. Epub 2021 Sep 20. 2021
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| 2 | LONP1, LONP2, TYSND1
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| The peroxisomal Lon protease LonP2 in aging and disease: functions and comparisons with mitochondrial Lon protease LonP1.
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| Pomatto LC, Raynes R, Davies KJ.
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| Biol Rev Camb Philos Soc 92(2):739-753. doi: 10.1111/brv.12253. Epub 2016 Feb 8. Review.
2017
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| 3 | CODAS, LONP1, MT-CO2
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| CODAS Syndrome Is Associated with Mutations of LONP1, Encoding Mitochondrial AAA(+) Lon Protease.
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| Strauss KA, Jinks RN, Puffenberger EG, 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 AM, Prasad C, Chudley AE, Lee IN, Suzuki CK.
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| Am J Hum Genet 96(1):121-35. doi: 10.1016/j.ajhg.2014.12.003. 2015
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| 4 | LONP1, SIRT3
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| Sirtuin 3 interacts with Lon protease and regulates its acetylation status.
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| Gibellini L, Pinti M, Beretti F, Pierri CL, Onofrio A, Riccio M, Carnevale G, De Biasi S, Nasi M, Torelli F, Boraldi F, De Pol A, Cossarizza A.
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| Mitochondrion itochondrion. 2014 Aug 13. pii: S1567-7249(14)00116-0. doi: 10.1016/j.mito.2014.08.001. [Epub ahead of print]
2014
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| 5 | LONP1
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| ATP-dependent Lon protease controls tumor bioenergetics by reprogramming mitochondrial activity.
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| Quirós PM, Español Y, Acín-Pérez R, Rodríguez F, Bárcena C, Watanabe K, Calvo E, Loureiro M, Fernández-García MS, Fueyo A, Vázquez J, Enríquez JA, López-Otín C.
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| Cell Rep 8(2):542-56. doi: 10.1016/j.celrep.2014.06.018. Epub 2014 Jul 10.
2014
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| 6 | LONP1, STAR
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| StAR enhances transcription of genes encoding the mitochondrial proteases involved in its own degradation.
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| Bahat A, Perlberg S, Melamed-Book N, Lauria I, Langer T, Orly J.
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| Mol Endocrinol 28(2):208-24. doi: 10.1210/me.2013-1275. Epub 2013 Jan 1.
2014
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| 7 | LONP1
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| Effect of Lon protease knockdown on mitochondrial function in HeLa cells.
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| Bayot A, Gareil M, Chavatte L, Hamon MP, L'Hermitte-Stead C, Beaumatin F, Priault M, Rustin P, Lombès A, Friguet B, Bulteau AL.
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| Biochimie 100:38-47. doi: 10.1016/j.biochi.2013.12.005. Epub 2013 Dec 17.
2014
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| 8 | LONP1, TFAM
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| Phosphorylation of human TFAM in mitochondria impairs DNA binding and promotes degradation by the AAA+ Lon protease.
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| Lu B, Lee J, Nie X, Li M, Morozov YI, Venkatesh S, Bogenhagen DF, Temiakov D, Suzuki CK.
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| Mol Cell 49(1):121-32. doi: 10.1016/j.molcel.2012.10.023. Epub 2012 Nov 29.
2013
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| 9 | ALAS1, LONP1
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| Lon peptidase 1 (LONP1)-dependent breakdown of mitochondrial 5-aminolevulinic acid synthase protein by heme in human liver cells.
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| Tian Q, Li T, Hou W, Zheng J, Schrum LW, Bonkovsky HL.
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| J Biol Chem 286(30):26424-30. Epub 2011 Jun 9.
2011
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| 10 | LONP1
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| Functional characterization of the promoter of the human Lon protease gene.
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| Pinti M, Gibellini L, De Biasi S, Nasi M, Roat E, O'Connor JE, Cossarizza A.
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| Mitochondrion 11(1):200-6. doi: 10.1016/j.mito.2010.09.010. Epub 2010 Oct 7.
2011
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| 11 | LONP1, TFAM
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| Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM).
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| Matsushima Y, Goto Y, Kaguni LS.
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| Proc Natl Acad Sci U S A 107(43):18410-5. Epub 2010 Oct 7.
2010
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| 12 | CODAS, LONP1
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| Fourth case of cerebral, ocular, dental, auricular, skeletal syndrome (CODAS), description of new features and molecular analysis.
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| Marlin S, Ducou Le Pointe H, Le Merrer M, Portnoi MF, Chantot S, Jonard L, Mantel-Guiochon A, Siffroi JP, Garabedian EN, Denoyelle F.
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| Am J Med Genet A 152A(6):1510-4. doi: 10.1002/ajmg.a.33242.
2010
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| 13 | CLPP, FRDA, LONP1
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| Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins.
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| Guillon B, Bulteau AL, Wattenhofer-Donzé M, Schmucker S, Friguet B, Puccio H, Drapier JC, Bouton C.
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| FEBS J 276(4):1036-47. doi: 10.1111/j.1742-4658.2008.06847.x. Epub 2008 Jan 12.
2009
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| 14 | LONP1
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| Thermodynamic characterization of specific interactions between the human Lon protease and G-quartet DNA.
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| Chen SH, Suzuki CK, Wu SH.
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| Nucleic Acids Res 36(4):1273-87. Epub 2008 Jan 3.
2008
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| 15 | LONP1
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| Towards the control of intracellular protein turnover: mitochondrial Lon protease inhibitors versus proteasome inhibitors.
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| Bayot A, Basse N, Lee I, Gareil M, Pirotte B, Bulteau AL, Friguet B, Reboud-Ravaux M.
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| Biochimie 90(2):260-9. Epub 2007 Oct 25. Review.
2008
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| 16 | LONP1
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| Roles for the human ATP-dependent Lon protease in mitochondrial DNA maintenance.
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| Lu B, Yadav S, Shah PG, Liu T, Tian B, Pukszta S, Villaluna N, Kutejová E, Newlon CS, Santos JH, Suzuki CK.
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| J Biol Chem 282(24):17363-74. Epub 2007 Apr 9.
2007
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| 17 | LONP1
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| Cleavage site selection within a folded substrate by the ATP-dependent lon protease.
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| Ondrovicová G, Liu T, Singh K, Tian B, Li H, Gakh O, Perecko D, Janata J, Granot Z, Orly J, Kutejová E, Suzuki CK.
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| J Biol Chem 280(26):25103-10. Epub 2005 May 3.
2005
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| 18 | LONP1
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| Downregulation of the human Lon protease impairs mitochondrial structure and function and causes cell death.
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| Bota DA, Ngo JK, Davies KJ.
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| Free Radic Biol Med 38(5):665-77.
2005
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| 19 | LONP1
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| DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate.
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| Liu T, Lu B, Lee I, Ondrovicová G, Kutejová E, Suzuki CK.
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| J Biol Chem 279(14):13902-10. Epub 2004 Jan 22.
2004
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| 20 | LONP1
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| Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism.
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| Bota DA, Davies KJ.
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| Nat Cell Biol 4(9):674-80.
2002
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| 21 | LONP1
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| The human LON protease binds to mitochondrial promoters in a single-stranded, site-specific, strand-specific manner.
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| Fu GK, Markovitz DM.
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| Biochemistry 37(7):1905-9. 1998
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| 22 | APLP2, CAMK2B, CPB1, ENO1, VWF, GLS, ITPKC, LONP1, MTFN, PDE2A, PPP2R2A, MAP2K1L, RAB5B, SCN2A, TST
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| Toward a cDNA map of the human genome.
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| Korenberg JR, et al.
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| Genomics 29 : 364-370. 1995
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| 23 | LONP1
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| Cloning and sequence analysis of cDNA for a human homolog of eubacterial ATP-dependent Lon proteases.
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| Amerik AYu, et al.
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| FEBS Lett 340(1-2):25-8 1994
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| 24 | LONP1
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| A human mitochondrial ATP-dependent protease that is highly homologous to bacterial Lon protease.
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| Wang N, et al.
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| Proc Natl Acad Sci U S A 90(23):11247-51 1993
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