My Bibliography

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de Lonlay P, Valnot I, Barrientos A, Gorbatyuk M, Tzagoloff A, Taanman JW, Benayoun E, Chrétien D, Kadhom N, Lombès A, de Baulny HO, Niaudet P, Munnich A, Rustin P, Rötig A. A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure. Nat Genet. 2001 Sep;29(1):57-60. doi: 10.1038/ng706. PubMed PMID: 11528392.

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Zhong H, Barrientos A. The zinc finger motif in the mitochondrial large ribosomal subunit protein bL36m is essential for optimal yeast mitoribosome assembly and function. Biochim Biophys Acta Mol Cell Res. 2024 Apr;1871(4):119707. doi: 10.1016/j.bbamcr.2024.119707. Epub 2024 Mar 16. PubMed PMID: 38493895; PubMed Central PMCID: PMC11009049.

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Zhong H, Janer A, Khalimonchuk O, Antonicka H, Shoubridge EA, Barrientos A. BOLA3 and NFU1 link mitoribosome iron-sulfur cluster assembly to multiple mitochondrial dysfunctions syndrome. Nucleic Acids Res. 2023 Nov 27;51(21):11797-11812. doi: 10.1093/nar/gkad842. PubMed PMID: 37823603; PubMed Central PMCID: PMC10681725.

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Zeng R, Smith E, Barrientos A. Yeast Mitoribosome Large Subunit Assembly Proceeds by Hierarchical Incorporation of Protein Clusters and Modules on the Inner Membrane. Cell Metab. 2018 Mar 6;27(3):645-656.e7. doi: 10.1016/j.cmet.2018.01.012. PubMed PMID: 29514071; PubMed Central PMCID: PMC5951612.

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Zambrano A, Fontanesi F, Solans A, de Oliveira RL, Fox TD, Tzagoloff A, Barrientos A. Aberrant translation of cytochrome c oxidase subunit 1 mRNA species in the absence of Mss51p in the yeast Saccharomyces cerevisiae. Mol Biol Cell. 2007 Feb;18(2):523-35. doi: 10.1091/mbc.e06-09-0803. Epub 2006 Nov 29. PubMed PMID: 17135289; PubMed Central PMCID: PMC1783774.

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Wiley DJ, Catanuto P, Fontanesi F, Rios C, Sanchez N, Barrientos A, Verde F. Bot1p is required for mitochondrial translation, respiratory function, and normal cell morphology in the fission yeast Schizosaccharomyces pombe. Eukaryot Cell. 2008 Apr;7(4):619-29. doi: 10.1128/EC.00048-07. Epub 2008 Feb 1. PubMed PMID: 18245278; PubMed Central PMCID: PMC2292624.

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Vempati UD, Diaz F, Barrientos A, Narisawa S, Mian AM, Millán JL, Boise LH, Moraes CT. Role of cytochrome C in apoptosis: increased sensitivity to tumor necrosis factor alpha is associated with respiratory defects but not with lack of cytochrome C release. Mol Cell Biol. 2007 Mar;27(5):1771-83. doi: 10.1128/MCB.00287-06. Epub 2007 Jan 8. PubMed PMID: 17210651; PubMed Central PMCID: PMC1820455.

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Valnot I, von Kleist-Retzow JC, Barrientos A, Gorbatyuk M, Taanman JW, Mehaye B, Rustin P, Tzagoloff A, Munnich A, Rötig A. A mutation in the human heme A:farnesyltransferase gene (COX10 ) causes cytochrome c oxidase deficiency. Hum Mol Genet. 2000 May 1;9(8):1245-9. doi: 10.1093/hmg/9.8.1245. PubMed PMID: 10767350.

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Tzagoloff A, Barrientos A, Neupert W, Herrmann JM. Atp10p assists assembly of Atp6p into the F0 unit of the yeast mitochondrial ATPase. J Biol Chem. 2004 May 7;279(19):19775-80. doi: 10.1074/jbc.M401506200. Epub 2004 Mar 3. PubMed PMID: 14998992.

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Tu YT, Barrientos A. The Human Mitochondrial DEAD-Box Protein DDX28 Resides in RNA Granules and Functions in Mitoribosome Assembly. Cell Rep. 2015 Feb 17;10(6):854-864. doi: 10.1016/j.celrep.2015.01.033. Epub 2015 Feb 13. PubMed PMID: 25683708; PubMed Central PMCID: PMC4534351.

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Timón-Gómez A, Nývltová E, Abriata LA, Vila AJ, Hosler J, Barrientos A. Mitochondrial cytochrome c oxidase biogenesis: Recent developments. Semin Cell Dev Biol. 2018 Apr;76:163-178. doi: 10.1016/j.semcdb.2017.08.055. Epub 2017 Sep 8. Review. PubMed PMID: 28870773; PubMed Central PMCID: PMC5842095.

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Timón-Gómez A, Barrientos A. Mitochondrial respiratory chain composition and organization in response to changing oxygen levels. J Life Sci (Westlake Village). 2020 Jun;2(2). doi: 10.36069/JoLS/20200601. PubMed PMID: 32551463; PubMed Central PMCID: PMC7302114.

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Timón-Gómez A, Pérez-Pérez R, Nyvltova E, Ugalde C, Fontanesi F, Barrientos A. Protocol for the Analysis of Yeast and Human Mitochondrial Respiratory Chain Complexes and Supercomplexes by Blue Native Electrophoresis. STAR Protoc. 2020 Sep 18;1(2). doi: 10.1016/j.xpro.2020.100089. Epub 2020 Sep 3. PubMed PMID: 32995753; PubMed Central PMCID: PMC7521667.

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Timón-Gómez A, Bartley-Dier EL, Fontanesi F, Barrientos A. HIGD-Driven Regulation of Cytochrome c Oxidase Biogenesis and Function. Cells. 2020 Dec 6;9(12). doi: 10.3390/cells9122620. Review. PubMed PMID: 33291261; PubMed Central PMCID: PMC7762129.

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Timón-Gómez A, Scharr AL, Wong NY, Ni E, Roy A, Liu M, Chau J, Lampert JL, Hireed H, Kim NS, Jan M, Gupta AR, Day RW, Gardner JM, Wilson RJA, Barrientos A, Chang AJ. Tissue-specific mitochondrial HIGD1C promotes oxygen sensitivity in carotid body chemoreceptors. Elife. 2022 Oct 18;11. doi: 10.7554/eLife.78915. PubMed PMID: 36255054; PubMed Central PMCID: PMC9635879.

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Tigano M, Ruotolo R, Dallabona C, Fontanesi F, Barrientos A, Donnini C, Ottonello S. Elongator-dependent modification of cytoplasmic tRNALysUUU is required for mitochondrial function under stress conditions. Nucleic Acids Res. 2015 Sep 30;43(17):8368-80. doi: 10.1093/nar/gkv765. Epub 2015 Aug 3. PubMed PMID: 26240381; PubMed Central PMCID: PMC4787798.

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Soto IC, Barrientos A. Mitochondrial Cytochrome c Oxidase Biogenesis Is Regulated by the Redox State of a Heme-Binding Translational Activator. Antioxid Redox Signal. 2016 Feb 20;24(6):281-98. doi: 10.1089/ars.2015.6429. Epub 2015 Nov 2. PubMed PMID: 26415097; PubMed Central PMCID: PMC4761835.

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Soto IC, Fontanesi F, Liu J, Barrientos A. Biogenesis and assembly of eukaryotic cytochrome c oxidase catalytic core. Biochim Biophys Acta. 2012 Jun;1817(6):883-97. doi: 10.1016/j.bbabio.2011.09.005. Epub 2011 Sep 16. Review. PubMed PMID: 21958598; PubMed Central PMCID: PMC3262112.

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Soto IC, Fontanesi F, Myers RS, Hamel P, Barrientos A. A heme-sensing mechanism in the translational regulation of mitochondrial cytochrome c oxidase biogenesis. Cell Metab. 2012 Dec 5;16(6):801-13. doi: 10.1016/j.cmet.2012.10.018. PubMed PMID: 23217259; PubMed Central PMCID: PMC3523284.

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Soto IC, Fontanesi F, Valledor M, Horn D, Singh R, Barrientos A. Synthesis of cytochrome c oxidase subunit 1 is translationally downregulated in the absence of functional F1F0-ATP synthase. Biochim Biophys Acta. 2009 Nov;1793(11):1776-86. doi: 10.1016/j.bbamcr.2009.09.002. Epub 2009 Sep 6. PubMed PMID: 19735676; PubMed Central PMCID: PMC2764804.

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Soto I, Couvillion M, Hansen KG, McShane E, Moran JC, Barrientos A, Churchman LS. Balanced mitochondrial and cytosolic translatomes underlie the biogenesis of human respiratory complexes. Genome Biol. 2022 Aug 9;23(1):170. doi: 10.1186/s13059-022-02732-9. PubMed PMID: 35945592; PubMed Central PMCID: PMC9361522.

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Solans A, Zambrano A, Barrientos A. Cytochrome c oxidase deficiency: from yeast to human. Preclinica. 2004; 2(5):336-348.

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Solans A, Zambrano A, Rodríguez M, Barrientos A. Cytotoxicity of a mutant huntingtin fragment in yeast involves early alterations in mitochondrial OXPHOS complexes II and III. Hum Mol Genet. 2006 Oct 15;15(20):3063-81. doi: 10.1093/hmg/ddl248. Epub 2006 Sep 12. PubMed PMID: 16968735.

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Santos B, Zeng R, Jorge SF, Ferreira-Junior JR, Barrientos A, Barros MH. Functional analyses of mitoribosome 54S subunit devoid of mitochondria-specific protein sequences. Yeast. 2022 Mar;39(3):208-229. doi: 10.1002/yea.3678. Epub 2021 Nov 10. PubMed PMID: 34713496; PubMed Central PMCID: PMC8969203.

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Ruetenik AL, Ocampo A, Ruan K, Zhu Y, Li C, Zhai RG, Barrientos A. Attenuation of polyglutamine-induced toxicity by enhancement of mitochondrial OXPHOS in yeast and fly models of aging. Microb Cell. 2016 Jul 26;3(8):338-351. doi: 10.15698/mic2016.08.518. PubMed PMID: 28357370; PubMed Central PMCID: PMC5349013.

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Ruetenik A, Barrientos A. Dietary restriction, mitochondrial function and aging: from yeast to humans. Biochim Biophys Acta. 2015 Nov;1847(11):1434-47. doi: 10.1016/j.bbabio.2015.05.005. Epub 2015 May 12. Review. PubMed PMID: 25979234; PubMed Central PMCID: PMC4575837.

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Ruetenik A, Barrientos A. Exploiting Post-mitotic Yeast Cultures to Model Neurodegeneration. Front Mol Neurosci. 2018;11:400. doi: 10.3389/fnmol.2018.00400. eCollection 2018. Review. PubMed PMID: 30450036; PubMed Central PMCID: PMC6224518.

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Pérez-Pérez R, Lobo-Jarne T, Milenkovic D, Mourier A, Bratic A, García-Bartolomé A, Fernández-Vizarra E, Cadenas S, Delmiro A, García-Consuegra I, Arenas J, Martín MA, Larsson NG, Ugalde C. COX7A2L Is a Mitochondrial Complex III Binding Protein that Stabilizes the III2+IV Supercomplex without Affecting Respirasome Formation. Cell Rep. 2016 Aug 30;16(9):2387-98. doi: 10.1016/j.celrep.2016.07.081. Epub 2016 Aug 18. PubMed PMID: 27545886; PubMed Central PMCID: PMC5007171.

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Pinkerton M, Ruetenik A, Bazylianska V, Nyvltova E, Barrientos A. Salvage NAD+ biosynthetic pathway enzymes moonlight as molecular chaperones to protect against proteotoxicity. Hum Mol Genet. 2021 May 17;30(8):672-686. doi: 10.1093/hmg/ddab080. PubMed PMID: 33749726.

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Perales-Clemente E, Bayona-Bafaluy MP, Pérez-Martos A, Barrientos A, Fernández-Silva P, Enriquez JA. Restoration of electron transport without proton pumping in mammalian mitochondria. Proc Natl Acad Sci U S A. 2008 Dec 2;105(48):18735-9. doi: 10.1073/pnas.0810518105. Epub 2008 Nov 19. PubMed PMID: 19020091; PubMed Central PMCID: PMC2585044.

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Paul MF, Barrientos A, Tzagoloff A. A single amino acid change in subunit 6 of the yeast mitochondrial ATPase suppresses a null mutation in ATP10. J Biol Chem. 2000 Sep 22;275(38):29238-43. doi: 10.1074/jbc.M004546200. PubMed PMID: 10867012.

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Patel N, Barrientos A, Landgraf R. The growth factor receptor ERBB2 regulates mitochondrial activity on a signaling time scale. J Biol Chem. 2013 Dec 6;288(49):35253-65. doi: 10.1074/jbc.M113.478271. Epub 2013 Oct 18. PubMed PMID: 24142693; PubMed Central PMCID: PMC3853274.

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Pang HW, Barrientos A. Rapid Cryopurification of the Yeast Mitochondrial Ribosome. Methods Mol Biol. 2023;2661:133-141. doi: 10.1007/978-1-0716-3171-3_9. PubMed PMID: 37166636; PubMed Central PMCID: PMC10654548.

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Pan Y, Schroeder EA, Ocampo A, Barrientos A, Shadel GS. Regulation of yeast chronological life span by TORC1 via adaptive mitochondrial ROS signaling. Cell Metab. 2011 Jun 8;13(6):668-78. doi: 10.1016/j.cmet.2011.03.018. PubMed PMID: 21641548; PubMed Central PMCID: PMC3110654.

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Ocampo A, Barrientos A. Developing yeast models of human neurodegenerative disorders. Methods Mol Biol. 2011;793:113-27. doi: 10.1007/978-1-61779-328-8_8. PubMed PMID: 21913097.

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Ocampo A, Liu J, Barrientos A. NAD+ salvage pathway proteins suppress proteotoxicity in yeast models of neurodegeneration by promoting the clearance of misfolded/oligomerized proteins. Hum Mol Genet. 2013 May 1;22(9):1699-708. doi: 10.1093/hmg/ddt016. Epub 2013 Jan 18. PubMed PMID: 23335597; PubMed Central PMCID: PMC3657478.

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Ocampo A, Barrientos A. Quick and reliable assessment of chronological life span in yeast cell populations by flow cytometry. Mech Ageing Dev. 2011 Jun-Jul;132(6-7):315-23. doi: 10.1016/j.mad.2011.06.007. Epub 2011 Jun 28. PubMed PMID: 21736893.

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Ocampo A, Liu J, Schroeder EA, Shadel GS, Barrientos A. Mitochondrial respiratory thresholds regulate yeast chronological life span and its extension by caloric restriction. Cell Metab. 2012 Jul 3;16(1):55-67. doi: 10.1016/j.cmet.2012.05.013. PubMed PMID: 22768839; PubMed Central PMCID: PMC3397320.

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Ocampo A, Zambrano A, Barrientos A. Suppression of polyglutamine-induced cytotoxicity in Saccharomyces cerevisiae by enhancement of mitochondrial biogenesis. FASEB J. 2010 May;24(5):1431-41. doi: 10.1096/fj.09-148601. Epub 2009 Dec 14. PubMed PMID: 20008543.

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Ocampo A, Barrientos A. From the bakery to the brain business: developing inducible yeast models of human neurodegenerative disorders. Biotechniques. 2008 Oct;45(4):vii-xiv. doi: 10.2144/000112746. PubMed PMID: 18855765.

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Nývltová E, Dietz JV, Seravalli J, Khalimonchuk O, Barrientos A. Coordination of metal center biogenesis in human cytochrome c oxidase. Nat Commun. 2022 Jun 24;13(1):3615. doi: 10.1038/s41467-022-31413-1. PubMed PMID: 35750769; PubMed Central PMCID: PMC9232578.

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Neal SE, Dabir DV, Tienson HL, Horn DM, Glaeser K, Ogozalek Loo RR, Barrientos A, Koehler CM. Mia40 Protein Serves as an Electron Sink in the Mia40-Erv1 Import Pathway. J Biol Chem. 2015 Aug 21;290(34):20804-20814. doi: 10.1074/jbc.M115.669440. Epub 2015 Jun 17. PubMed PMID: 26085103; PubMed Central PMCID: PMC4543643.

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Moreno-Lastres D, Fontanesi F, García-Consuegra I, Martín MA, Arenas J, Barrientos A, Ugalde C. Mitochondrial complex I plays an essential role in human respirasome assembly. Cell Metab. 2012 Mar 7;15(3):324-35. doi: 10.1016/j.cmet.2012.01.015. Epub 2012 Feb 16. PubMed PMID: 22342700; PubMed Central PMCID: PMC3318979.

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Moraes CT, Diaz F, Barrientos A. Defects in the biosynthesis of mitochondrial heme c and heme a in yeast and mammals. Biochim Biophys Acta. 2004 Dec 6;1659(2-3):153-9. doi: 10.1016/j.bbabio.2004.09.002. Review. PubMed PMID: 15576047.

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Moraes CT, Dey R, Barrientos A. Transmitochondrial technology in animal cells. Methods Cell Biol. 2001;65:397-412. doi: 10.1016/s0091-679x(01)65023-4. PubMed PMID: 11381606.

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Moorthy BT, Jiang C, Patel DM, Ban Y, O'Shea CR, Kumar A, Yuan T, Birnbaum MD, Gomes AV, Chen X, Fontanesi F, Lampidis TJ, Barrientos A, Zhang F. The evolutionarily conserved arginyltransferase 1 mediates a pVHL-independent oxygen-sensing pathway in mammalian cells. Dev Cell. 2022 Mar 14;57(5):654-669.e9. doi: 10.1016/j.devcel.2022.02.010. Epub 2022 Mar 4. PubMed PMID: 35247316; PubMed Central PMCID: PMC8957288.

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Miró O, Barrientos A, Alonso JR, Casademont J, Jarreta D, Urbano-Márquez A, Cardellach F. Effects of general anaesthetic procedures on mitochondrial function of human skeletal muscle. Eur J Clin Pharmacol. 1999 Mar;55(1):35-41. doi: 10.1007/s002280050589. PubMed PMID: 10206082.

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Miró O, Cardellach F, Barrientos A, Casademont J, Rötig A, Rustin P. Cytochrome c oxidase assay in minute amounts of human skeletal muscle using single wavelength spectrophotometers. J Neurosci Methods. 1998 Mar 13;80(1):107-11. doi: 10.1016/s0165-0270(97)00204-5. PubMed PMID: 9606056.

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Miró O, Jarreta D, Casademont J, Barrientos A, Rodríguez B, Gómez M, Nunes V, Urbano-Márquez A, Cardellach F. Absence of mitochondrial dysfunction in polymyalgia rheumatica. Evidence based on a simultaneous molecular and biochemical approach. Scand J Rheumatol. 1999;28(5):319-23. doi: 10.1080/03009749950155526. PubMed PMID: 10568430.

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Miró O, Casademont J, Barrientos A, Urbano-Márquez A, Cardellach F. Mitochondrial cytochrome c oxidase inhibition during acute carbon monoxide poisoning. Pharmacol Toxicol. 1998 Apr;82(4):199-202. doi: 10.1111/j.1600-0773.1998.tb01425.x. PubMed PMID: 9584335.