Juried or Refereed Journal Articles

Public URL for “my bibliography” collection in My NCBI is: http://www.ncbi.nlm.nih.gov/sites/myncbi/antoni.barrientos.1/bibliography/41138774/public/?sort=date&direction=ascending. There you can find a complete list of publications.

  1. Lobo-Jarne T, Nývltová E, Pérez-Pérez R, Timón-Gómez A, Molinié T, Choi A, Mourier A, Fontanesi F, Ugalde C, and Barrientos AHuman COX7A2L regulates the biogenetic rates of respiratory chain complex III and complex III-containing supercomplexes Cell Reports (2018) In press
  2. Maiti P, Kim H-J, Tu Y-T and Barrientos A.Human GTPBP10 is essential for mitochondrial ribosome maturation. Nucleic Acids Res. (2018) In press
  3. Kim H-J, Barrientos A. MTG1 Couples Mitoribosome Large Subunit Assembly with Inter-Subunit Bridge Formation. Nucleic Acids Res. 46(16):8435-8453 (2018)
  4. 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 27(3):645-656 (2018)
  5. Guedes-Monteiro RF, Ferreira-Junior JR, Bleicher L, Nóbrega FG, Barrientos A, Barros MH. Mitochondrial ribosome bL34 mutants present diminished translation of cytochrome oxidase subunits. Cell Biol Int.42(6):630-642 (2018)
  6. Carmona-Gutierrez D, Bauer MA, Zimmermann A, Aguilera A, Austriaco N, Ayscough K, Balzan R, Bar-Nun S,Barrientos A, Belenky P et al. Guidelines and recommendations on yeast cell death nomenclature. Microb Cell. 5(1):4-31 (2018)
  7. Burstein S.R. Valsecchi F., Kawamata H., Bourens M., Zeng R., Zuberi A., Milner T.A., Cloonan S.M., Lutz C., Barrientos A.and G Manfredi G. In vitro and in vivo studies of the ALS-FTLD protein CHCHD10 reveal novel mitochondrial topology and protein interactions. Human Molecular Genetics, 27(1):160-177 (2018)
  8. Timon-Gomez A., Nyvltova E., Abriata L.A., Vila A., Hosler J and Barrientos A.Mitochondrial cytochrome oxidase biogenesis: recent developments. Semin Cell and Dev Biol76:163-178 (2017)
  9. De Silva D, PoliquinS, Zeng R, Zamudio-Ochoa A, Marrero N, Perez-Martinez X, Fontanesi F, Barrientos A. The DEAD-box helicase Mss116 plays distinct roles in mitochondrial ribogenesis and mRNA-specific translation. Nucleic Acids Res.45(11):6628-6643 (2017)
  10. Kim HJ, Maiti P, Barrientos A.Mitochondrial ribosomes in cancer. Semin Cancer Biol. 47:67-81 (2017)
  11. Bourens M and Barrientos A. Human mitochondrial cytochrome c oxidase assembly factor COX18 acts transiently as a membrane insertase within the subunit 2 maturation module. J Biol Chem.292(19):7774-7783 (2017)
  12. Bourens M and Barrientos A. A TALEN-mediated CMC1-Knockout cell line reveals translation-independent control of human mitochondrial Complex IV biogenesis. EMBO Reports. 18(3):477-494 (2017)
  13. Kumar A, Birnbaum MD, Patel DM, Morgan WM, Singh J, Barrientos A, Zhang F. Posttranslational arginylation enzyme Ate1 affects DNA mutagenesis by regulating stress response.Cell Death Dis.7(9):e2378 (2016)
  14. Ruetenik AL, Ocampo A, RuanK, ZhuY, LiC, R. ZhaiGR and Barrientos A.Attenuation of polyglutamine-induced toxicity by enhancement of mitochondrial OXPHOS in yeast and fly models of aging. Microbial Cell, 3 (8):338-51 (2016)
  15. Bohovych I, Kastora S, Christianson S, Topil D, Kim H, Fangman T, Zhou YJ, Barrientos A, Lee J, Brown AJ, Khalimonchuk O. Oma1 Links MitochondrialProtein Quality Control and TOR Signaling To Modulate Physiological Plasticity and Cellular Stress Responses. Cell Biol.36(17):2300-12 (2016)
  16. Soto I and Barrientos A.Cytochromecoxidase biogenesis is regulated by the redox state of a heme-binding translational activator.Antiox Redox Signal24(6):281-98(2015)
  17. Abrams AJ, Hufnagel RB, Rebelo A, Zanna C, Patel N, Gonzalez MA, Campeanu IJ, Griffin LB, Groenewald S, Strickland AV, Tao F, Speziani F, Abreu L, Schüle R, Caporali L, La Morgia C, Maresca A, Liguori R, Lodi R, Ahmed ZM, Sund KL, Wang X, Krueger LA, Peng Y, Prada CE, Prows CA, Schorry EK, Antonellis A, Zimmerman HH, Abdul-Rahman OA, Yang Y, Downes SM, Prince J, Fontanesi F, Barrientos A, Németh AH, Carelli V, Huang T, Zuchner S, Dallman JE. Mutations in SLC25A46, encoding a UGO1-like protein, cause an optic atrophy spectrum disorder. Nat Genet. 2015 47(8):926-32 (2015)
  18. Tigano M, Ruotolo R, Dallabona C, Fontanesi F, Barrientos A, Donnini C, and Ottonello S. (2015). Elongator-dependent modification of cytoplasmic tRNALysUUUis required for mitochondrial function under stress conditions. Nucleic Acids Res. 43(17):8368-80 (2015).
  19. Barrientos A. Mitochondriolus: assembling mitoribosomes 2015 6(19):16800-1 (2015)
  20. Neal SE, Dabir DV, Tienson HL, Horn DM, Glaeser K, Ogozalek Loo RR, Barrientos A, Koehler CM. Mia40 serves as an electron sink in the Mia40-Erv1 import pathway.J Biol Chem. 290(34):20804-14 (2015)
  21. De Silva D, Tu YT, Amunts A, Fontanesi F, Barrientos A. (2015) Mitochondrial ribosome assembly in health and disease. Cell Cycle. 14(14):2226-50
  22. Ruetenik AL and Barrientos A. (2015) Dietary restriction, mitochondrial function and aging: from yeast to humans. BBA: Bioenergetics. 1847 (2015) 14341447
  23. Tu YT and Barrientos A. The human mitochondrial DEAD-Box protein DDX28 resides in RNA granules and functions in mitoribosome assembly. Cell Reports. 10: 854-64 (2015)
  24. Hess KC, Liu J, Manfredi G, Mühlschlegel FA, Buck J, Levin LR, Barrientos A. A mitochondrial CO2-adenylyl cyclase-cAMP signalosome controls yeast normoxic cytochrome oxidase activity. FASEB J.28: 4369-80 (2014)
  25. Bourens M, Boulet A, Leary SC, Barrientos A.Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome oxidase. Mol. Genet. 23(11): 2901-13 (2014)
  26. De Silva D, Fontanesi F and Barrientos A. The DEAD-Box protein Mrh4 functions in the assembly of the mitochondrial large ribosomal subunit. Cell Metab. 18: 712-25 (2013)
  27. Patel N, Barrientos A, Landgraf R. The Growth Factor Receptor ERBB2 regulates Mitochondrial Activity on a Signaling Timescale. Biol. Chem. 288:35253-65 (2013)
  28. Samuli Kursu VA, Pietikäinen LP, Fontanesi F, Aaltonen MJ, Suomi F, Raghavan Nair R, Schonauer MS, Dieckmann CL, Barrientos A, Hiltunen JK, Kastaniotis AJ. Defects in mitochondrial fatty acid synthesis result in failure of multiple aspects of mitochondrial biogenesis in Saccharomyces cerevisiae. Microbiol. 90: 824-40 (2013)
  29. Lu G, Matsuura SE, Barrientos A, Scott WA. HIV-1 Infection Is Blocked at an Early Stage in Cells Devoid of Mitochondrial DNA. PLoS One. 2013 8(10):e78035 (2013)
  30. Barrientos Aand Ugalde C. I function, therefore I am: overcoming doubt about mitochondrial supercomplexes. Cell Metab.July 18(2):147-9 (2013)
  31. Ocampo A, Liu JJ and Barrientos A.NAD+salvage pathway proteins suppress proteotoxicity in yeast models of neurodegeneration. Mol. Genet.22(9):1699-708 (2013)
  32. Clemente P, Peralta S, Cruz-Bermudez A, Echevarria L, Fontanesi F, Barrientos A, Fernandez-Moreno MA, Garesse R.hCOA3 stabilizes COX1 and promotes cytochrome oxidase assembly in human mitochondria. Biol. Chem.288(12):8321-31 (2013)
  33. Bourens M, Fontanesi F, Soto IC, Liu J, and Barrientos A.Reactive Oxygen Species and Redox Regulation of Mitochondrial Cytochrome Oxidase Biogenesis. Redox Signal.19(16):1940-52 (2013)
  34. Liu J and Barrientos A. Transcriptional regulation of yeast OXPHOS hypoxic genes by oxidative stress. RedoxSignal. 19(16):1916-27 (2013)
  35. Soto IC, Fontanesi F, Myers RS, Hamel P and Barrientos A.A Heme-Sensing Mechanism in the Translational Regulation of Mitochondrial Cytochrome c Oxidase Biogenesis. Cell Metab.16 (6): 801-813 (2012)
  36. Barrientos A. Complementary roles of mitochondrial respiration and ROS signaling on cellular aging and longevity.Aging4(9): 1-2 (2012)
  37. Bourens M, Dabir DV, Tienson HL, Sorokina, I, Koehler CM and Barrientos A.Role of twin-CysX9Cys motif cysteines in mitochondrial import of the cytochrome oxidase biogenesis factor Cmc1. J Biol Chem287(37):31258-69 (2012)
  38. Ocampo A, Liu JJ, Schroeder EA, Shadel GS and Barrientos A. Mitochondrial respiratory thresholds regulate yeast chronological life span and its extension by caloric restriction. Cell Metab.16(1):55-67 (2012)
  39. 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. 15(3):324-35 (2012)
  40. Soto IC, Fontanesi F, Liu J, Barrientos Biogenesis and assembly of eukaryotic cytochrome oxidase catalytic core.Biochim. Biophys. Acta. 1817(6):883-97 (2012)
  41. Ocampo A and Barrientos A.Developing yeast models of human neurodegenerative disorders. Methods Mol. Biol.793: 113-127 (2011)
  42. Yong Pan Y, Schroeder EA, Ocampo A, Barrientos Aand Shadel GS. Regulation of yeast chronological life span by TORC1 via adaptive mitochondrial ROS signaling. Cell Metab. 13(6): 668-678 (2011)
  43. Ocampo A and Barrientos A. Quick and reliable assessment of chronological life span in yeast cell populations by flow cytometry. Ageing. Dev.132:315-323(2011)
  44. Fontanesi F, Clemente P, Barrientos A.Cox25 teams up with Mss51, Ssc1 and Cox14 to regulate mitochondrial cytochrome oxidase subunit 1 expression and assembly in Saccharomyces cerevisiae. Biol. Chem. 286(1):255-266 (2011)
  45. Horn D, Zhou W, Trevisson E, Al-Ali H, Harris TK, Salviati L, and Barrientos A.The conserved mitochondrial twin CX9C protein Cmc2 is a Cmc1 homologue essential for cytochrome oxidase biogenesis. Biol. Chem. 285(20):15088-99(2010)
  46. Fontanesi F., Soto I.C., Horn D., Barrientos A.Mss51 and Ssc1 facilitate translational regulation of cytochrome oxidase biogenesis. Mol Cell Biol. 30: 245-259 (2010)
  47. Ocampo A, Zambrano A. and Barrientos A.Suppression of polyglutamine-induced cytotoxicity in Saccharomyces cerevisiae by enhancement of mitochondrial biogenesis. FASEB J.24(5):1431-41(2010)
  48. Soto IC, Fontanesi F, Valledor M, Horn D, Singh R. and Barrientos A. Synthesis of cytochrome oxidase subunit 1 is translationally down-regulated in the absence of functional F1F0-ATP synthase. Biophys. Acta(Mol. Cell. Res.) 1793:1776-86 (2009)
  49. Barrientos A, Gouget K, Horn D, Soto IC and Fontanesi F. Suppression mechanisms of COX assembly defects in yeast and human: Insights into the COX assembly process. Biophys. Acta. (Mol. Cell. Res.) 1793:97-107(2009)
  50. Barrientos A., Fontanesi F. and Díaz F. Evaluation of the Mitochondrial Respiratory Chain and Oxidative Phosphorylation System using Polarography and Spectrophotometric Enzyme Assays. Curr Protoc Hum Genet. Chapter 19: Unit 19.3 (2009)
  51. Fontanesi F., Diaz F. and Barrientos A.Evaluation of the Mitochondrial Respiratory Chain and Oxidative Phosphorylation System Using Yeast Models of OXPHOS Deficiencies. Curr Protoc Hum Genet. Chapter 19: Unit 19.5 (2009)
  52. Díaz F., Barrientos A.and Fontanesi F. Evaluation of the Mitochondrial Respiratory Chain and Oxidative Phosphorylation System Using Blue Native Gel Electrophoresis.Curr Protoc Hum Genet. Chapter 19: Unit 19.4 (2009)
  53. Perales-Clemente E, Bayona-Bafaluy P, Pérez-Martos A, Barrientos A., Fernández-Silva P, and Enríquez JA. Restoration of electron transport without proton pumping in mammalian mitochondria. N.A.S105: 18735-18739 (2008)
  54. Ocampo A and Barrientos A.From the bakery to the brain business: developing inducible yeast models of human neurodegenerative disorders. Biotechniques45(4):Pvii-xiv (2008)
  55. Horn D, Al-Ali H, and Barrientos A.Cmc1p is a conserved mitochondrial twin Cx9C protein Involved in cytochrome oxidase biogenesis. Cell. Biol.28:4354-64(2008)
  56. Horn D and Barrientos A.Mitochondrial copper metabolism and delivery to cytochromecIUBMB Life. 60:421-9 (2008)
  57. Fontanesi F, Soto IC and Barrientos A.Cytochrome oxidase biogenesis: new levels of regulation. IUBMB Life. 60:557-68(2008)
  58. Fontanesi F., Jin C., Tzagoloff A., and Barrientos A.Transcriptional activators HAP/NF-Y rescue a cytochrome oxidase defect in yeast and human cells.Hum Mol Genet.17:775-88(2008)
  59. Dave K.R., DeFazio R.A., Raval AP, Torraco A, Saul I, Barrientos Aand Perez-Pinzon M.A.Ischemic preconditioning targets the respiration of synaptic mitochondria via protein kinase Epsilon. Neurosc.28: 4172-4182(2008)
  60. Wiley DJ, Catanuto P, Fontanesi F, Rios C, Sanchez N, Barrientos, and Verde F. Bot1p is required for mitochondrial translation, respiratory function and normal cell morphology in the fission yeast Schizosaccharomyces pombe. Eukaryotic Cell, 7: 619-29(2008)
  61. Horn D., Fontanesi F., and Barrientos A.Exploring protein-protein interactions involving newly synthesized mitochondrial DNA encoded proteins. Methods in Molecular Biology: Membrane Trafficking. 457. Chapter 9. Pg. 125-139. Ales Vancura, ed. Humana Press (2008)
  62. Gouget K., Verde F. and Barrientos A. In VivoLabeling and analysis of mitochondrial translation products in budding and in fission yeasts. Methods in Molecular Biology: Membrane Trafficking. 457. Chapter 8. Pg. 113-124. Ales Vancura, ed. Humana Press (2008)
  63. Jin C, Barrientos A, Epstein CB, Butow RA, and Tzagoloff A. SIT4regulation of Mig1p-mediated catabolite repression in Saccharomyces cerevisiae.FEBS Lett. 581:5658-63(2007)
  64. Zambrano A, Fontanesi F., Solans A., de Oliveira RL, Fox TD, Tzagoloff A, Barrientos A. Aberrant translation of cytochrome oxidase subunit 1 mRNA species in the absence of Mss51p in the yeast Saccharomyces cerevisiae. Biol. Cell.18:523-35. (2007)
  65. Vempati UD, Diaz F, Barrientos A, Narisawa S, Mian AM, Millan JL, Boise LH, Moraes C.T.Role of cytochrome in apoptosis: increased sensitivity to tumor necrosis factor alpha is associated with respiratory defects but not with lack of cytochrome release. Cell Biol.27(5):1771-83. (2007)
  66. Solans A., Zambrano A., Rodriguez M., and Barrientos A.Cytotoxicity of a mutant huntingtin fragment in yeast involves early alterations in mitochondrial OXPHOS complex II and III. Mol. Genet.,15(20):3063-81. (2006)
  67. Fontanesi F, Soto IC, Horn D, and Barrientos A.Assembly of mitochondrial cytochrome oxidase, a complicated and highly regulated cellular process. J. Physiol. – Cell Physiol., 291(6):C1129-47. (2006)
  68. Moraes CT, Diaz F, and Barrientos A. Defects in the biosynthesis of mitochondrial heme and heme in yeast and mammals Biophys. Acta – Bioenergetics, 1659: 153-159. (2005)
  69. Barrientos A, Zambrano A., and Tzagoloff A.Mss51p and Cox14p jointly regulate mitochondrial Cox1p expression in Saccharomyces cerevisiae. EMBO J. 23(17):3472-82. (2004)
  70. Solans A, Zambrano A, and Barrientos A.Cytochromecoxidase deficiency: from yeast to human Preclinica. 2(5):336-348 (2004)
  71. Tzagoloff A, Barrientos A, Neupert W, Herrmann JM.Atp10p assists assembly of Atp6p into the F0unit of the yeast mitochondrial ATPase Biol. Chem. 279: 19775-80(2004)
  72. Barrientos A.Yeast models of human mitochondrial diseasesIUBMB Life. 55:83-95. (2003)
  73. Barrientos A, Korr D, Barwell KJ, Christian Sjulsen C, Gajewski CD, Manfredi G, Sharon Ackerman S, and Tzagoloff A. MTG1codes for a conserved protein required for mitochondrial translation Biol. Cell14:2292-302. (2003)
  74. Jin C, Barrientos A, and Tzagoloff A. Yeast dihydroxybutanone phosphate synthase, an enzyme of the riboflavin biosynthetic pathway has a second unrelated function in expression of mitochondrial respiration Biol. Chem.278:14698-703. (2003)
  75. Barrientos A, Pierre D, Lee J, and Tzagoloff A. Cytochrome oxidase assembly does not require catalytically active cytochrome J. Biol. Chem.278:8881-7. (2003)
  76. Carlson C.G., Barrientos A., Tzagoloff A., and Glerum D.M. COX16encodes a novel protein required for the assembly of cytochrome oxidase in Saccharomyces cerevisiae Biol. Chem. 278: 3770-5. (2002)
  77. Ludovico P, Rodrigues F, Almeida A, Silva MT, Barrientos A.and Côrte-Real, M.Cytochrome crelease and mitochondria involvement in programmed cell death induced by acetic acid inSaccharomyces cerevisiaeBiol. Cell13:2598-606. (2002)
  78. Barrientos A.In vivo and in organello assessment of OXPHOS activities A companion of Methods Enzymol.26: 307-16. (2002)
  79. Barrientos A, Barros M, Valnot I, Rotig A, Rustin P, and Tzagoloff A. Cytochrome oxidase in health and disease. Gene. 286(1): 53-63. (2002)
  80. Barrientos A, Korr D, and Tzagoloff A.Mitochondrial cytochrome c oxidase assembly: Shy1p is necessary for full expression of subunit 1 in the yeast model of Leigh’s syndrome EMBO J.21(1-2): 43-52. (2002)
  81. D’Aurelio M, Pallotti F, Barrientos A, Gajewski CD, Kwong JQ, Bruno C, Beal MF, Manfredi G. In vivo regulation of oxidative phosphorylation in cells harboring a stop-codon mutation in mitochondrial DNA-encoded cytochrome c oxidase subunit I Biol. Chem. 276(50): 46925-32. (2001)
  82. de Lonlay P, Valnot I, Barrientos A, Gorbatyuk, TzagoloffA, Benayoun E, ChrétienD, KadhomN, LombèsA, Ogier de BaulnyH, NiaudetP, Rustin P, Munnich A and Rötig A Mutations in BCS1, a mitochondral respiratory chain assembly gene, are responsible for the complex III deficiency of patients with fatal tubulophathy and liver failure Nat. Genet. 29: 57-60.(2001)
  83. Barrientos A, DeyR, Müller S,Wienberg J, and Moraes CT. Cytochrome oxidase assembly in primates is extremely sensitive to evolutionary variations in amino acid sequence Biol. Evol. 17: 1508-1519.(2000)
  84. 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 Biol. Chem. 275: 29238-43.(2000)
  85. Dey R., Barrientos A. and Moraes C.T. Functional Constraints of Nuclear – Mitochondrial DNA Interactions in Xenomitochondrial Rodent Cell Lines Biol. Chem. 275:31520-31527.(2000)
  86. Valnot I., Kleist-Retzow J.-C., Barrientos A., Gorbatyuk M., Rustin P., Tzagoloff A., Munnich A., and Rötig A. Mutation in the haem A: Farnesyl transferase (COX10) gene in cytochrome c oxidase deficiency Mol. Genet.9:1245-9.(2000)
  87. Jarreta D., Orus J., Barrientos A., Miró O, Roig E., Heras M., Moraes, C.T., Cardellach F. and Casademont J. Mitochondrial function in heart muscle from patients with idiopathic dilated cardiomyopathy Res. 45:860-5. (2000)
  88. Barrientos A.and Moraes C.T. Titrating the effects of mitochondrial complex I impairment in the cell physiology Biol. Chem. 274:16188-97. (1999)
  89. Miró O., Barrientos A., Alonso J.R., Casademont J., Jarreta, D. Urbano-Márquez, A., and Cardellach F. Effects of general anaesthetic procedures on mitochondrial function of human skeletal muscle J. Clin. Pharmacol. 55(1):35-41. (1999)
  90. Biarnes J., Barrientos A., Ricart W., Nunes V., Fernandez-Castaner M., and Soler J.[Diabetes mellitus associated with the A3243G mutation of mitochondrial DNA. Apropos a case] Clin. (Barc)112(3):99-101. (1999)
  91. Gomez Zaera M., Barrientos A., Arias L., Rojas I., Arruga J., Estivill X., Casademont, J., Nunes, V.[An analysis of 6 Leber mutations in 31 individuals with optic atrophy. A study of its transmission in 5 families] Clin. (Barc) 112:326-329. (1999)
  92. Miró O., Jarreta D., Casademont J., Barrientos A., Rodríguez B., Gomez M., Nunes V., Urbano-Márquez A. and Cardellach F. Absence of mitochondrial dysfunction in polymyalgia rheumatica. Evidence based on a simultaneous molecular and biochemical approach J. Rheumatol.28(5):319-23 (1999)
  93. Barrientos Aand Moraes C.T. Simultaneous transfer of mitochondrial DNA and single chromosomes in somatic cells: a novel approach for the study of defects in nuclear-mitochondrial communication Mol. Genet.7: 1801-1808 (1998)
  94. Barrientos A.,Kenyon L.and Moraes C.T. Human xenomitochondrial cybrids: cellular models of mitochondrial complex I deficiency Biol. Chem. 273: 14210-14217. (1998)
  95. Masanés F., Barrientos A., Cebrian M., Pedrol E., Miró O., Casademont J., and Grau J.M. Clinical, histological and molecular reversibility of zidovudine myopathy Neurol. Sci. 159: 226-228. (1998)
  96. Barrientos A., Marín C., Miró O., Casademont J., Gómez M., Nunes V., Tolosa E., Urbano-Márquez A., and Cardellach F. Biochemical and molecular effects of chronic haloperidol administration on brain and muscle mitochondria of rats Neurosci. Res. 53: 475-481. (1988)
  97. Miró O., Casademont J., Barrientos A., Urbano-Márquez A., and Cardellach F. Mitochondrial cytochrome c oxidase inhibition during acute carbon monoxide poisoning Toxicol.82: 199-202. (1998)
  98. Miró O., Cardellach F., Barrientos A., Casademont, J., Rötig A., and Rustin P. Cytochrome oxidase assay in minute amount of human skeletal muscle mitochondria using simple wavelength spectrophotometers J Neurosci Methods. 80: 107-111. (1998)
  99. Chretien D., Gallego J., Barrientos A., Casademont J., Cardellach F., Rötig A., Munnich A. and Rustin, P. Biochemical parameters for the diagnosis of respiratory chain deficiency in humans and their lack of age-related changes J. 329: 249-254 (1998)
  100. Barrientos A., Casademont J., Cardellach F., Estivill X, Urbano-Márquez A., and Nunes V. Reduced steady-state levels of mitochondrial RNA and increased mitochondrial DNA amount in human brain with ageing Brain Res.52: 284-289(1997)
  101. Barrientos A., Casademont J., Cardellach F., Ardite E., Estivill E., Urbano-Márquez A., Fernández-Checa J.C. and Nunes V. Qualitative and quantitative changes in skeletal muscle mtDNA and expression of mitochondrial-encoded genes in the human aging process Mol. Med. 62, 165-171 (1997)
  102. Barrientos A., Casademont J., Genís D., Cardellach F., Fernández-Real J. M., Grau J.M., Urbano-Márquez A., Estivill X. and Nunes V. Sporadic heteroplasmic single 5.5 Kb mitochondrial DNA deletion associated with cerebellar ataxia, hypogonadotropic hypogonadism, choroidal dystrophy, and mitochondrial respiratory chain complex I deficiency Mut.10: 212-216 (1997)
  103. Conget I., Barrientos A., Manzanares J.M., Casademont J., Viñas O., Barceló J., Nunes V., Gomis R. and Cardellach, F. Respiratory chain activity and mitochondrial DNA content of non-purified and purified pancreatic islet cells Metabolism46: 984-987(1997)
  104. Barrientos A.,Casademont J., Rötig A., Miro O., Urbano-Marquez A., Rustin P., and Cardellach F. Absence of relationship between the level of electron transport chain activities and ageing in human skeletal muscle Biophys. Res. Commun. 229: 536-539 (1996)
  105. Barrientos A., Casademont J., Saiz A., Cardellach F., Volpini V., Solans A., Tolosa E., Urbano-Márquez A., Estivill X., and Nunes V. Autosomal recessive Wolfram syndrome associated with a 8.5-kb mtDNA single deletion J. Hum. Genet.58: 963-970 (1996)
  106. Barrientos A.,Volpini V., Casademont J., Genis D., Manzanares J.M., Ferrer I., Corral J., Cardellach F., Urbano-Márquez A., Estivill X., and Nunes V. A nuclear defect in the 4p16 region predisposes to multiple mitochondrial DNA deletions in families with Wolfram syndrome Clin. Invest. 97: 1570-1576 (1996)
  107. Casademont J., Barrientos A., Grau J.M., Pedrol E., Estivill X., Urbano-Márquez A. and Nunes V. The effect of zidovudine on skeletal muscle mtDNA in HIV-1 infected patients with mild or no muscle dysfunction Brain 119: 1357- 1364 (1996)
  108. Conget I., Manzanares J.M., Barrientos A., Cardellach F. and Gomis R. Coenzyme Q and insuline secretion in vitroDiabetes Res. Clin. Pract. 33: 135-136 (1996)
  109. Barrientos A.,Casademont J., Grau J.M., Cardellach F., Montoya J., Estivill X., Urbano-Márquez A. and Nunes V. Progressive external ophtalmoplegia and Kearns-Sayre syndrome: a clinical and molecular study of 6 cases Clin. (Barcelona)105: 180-184 (1995)
  110. Barrientos A.,Casademont J., Solans A., Moral P., Cardellach F., Urbano-Márquez A., Estivill X., and Nunes V. The 9-bp deletion in region V of mitochondrial DNA: evidence of mutation recurrence Genet.96: 225-228 (1995)
  111. Casademont I., Barrientos A., Cardellach F., Rötig A., Grau J.M., Montoya J., Beltrán B., Cervantes F., Rozman C., Estivill X., Urbano-Márquez A. and Nunes V. Multiple deletions in mtDNA in two brothers with sideroblastic anemia and mitochondrial myopathy and in their asymptomatic mother Mol. Genet.3: 1945-1949 (1994)