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Casademont J, Barrientos A, Cardellach F, Rötig A, Grau JM, Montoya J, Beltrán B, Cervantes F, Rozman C, Estivill X. Multiple deletions of mtDNA in two brothers with sideroblastic anemia and mitochondrial myopathy and in their asymptomatic mother. Hum Mol Genet. 1994 Nov;3(11):1945-9. doi: 10.1093/hmg/3.11.1945. PubMed PMID: 7874110.
Barrientos A, Casademont J, Grau JM, Cardellach F, Montoya J, Estivill X, Urbano-Márquez A, Nunes V. [Progressive external ophthalmoplegia and the Kearns-Sayre syndrome: a clinical and molecular study of 6 cases]. Med Clin (Barc). 1995 Jul 1;105(5):180-4. PubMed PMID: 7630231.
Barrientos A, Casademont J, Solans A, Moral P, Cardellach F, Urbano-Márquez A, Estivill X, Nunes V. The 9-bp deletion in region V of mitochondrial DNA: evidence of mutation recurrence. Hum Genet. 1995 Aug;96(2):225-8. doi: 10.1007/bf00207385. PubMed PMID: 7635476.
Cardellach F, Barrientos A, Miro O, Casademont J. Mitochondrial pathology and disease . In: HTA-Internal Medicine. Barcelona: Medicina Clinica; 1996. Chapter 2. . 501-545p.
Barrientos A, Volpini V, Casademont J, Genís D, Manzanares JM, Ferrer I, Corral J, Cardellach F, Urbano-Márquez A, Estivill X, Nunes V. A nuclear defect in the 4p16 region predisposes to multiple mitochondrial DNA deletions in families with Wolfram syndrome. J Clin Invest. 1996 Apr 1;97(7):1570-6. doi: 10.1172/JCI118581. PubMed PMID: 8601620; PubMed Central PMCID: PMC507219.
Barrientos A, Casademont J, Saiz A, Cardellach F, Volpini V, Solans A, Tolosa E, Urbano-Marquez A, Estivill X, Nunes V. Autosomal recessive Wolfram syndrome associated with an 8.5-kb mtDNA single deletion. Am J Hum Genet. 1996 May;58(5):963-70. PubMed PMID: 8651280; PubMed Central PMCID: PMC1914608.
Casademont J, Barrientos A, Grau JM, Pedrol E, Estivill X, Urbano-Márquez A, Nunes V. The effect of zidovudine on skeletal muscle mtDNA in HIV-1 infected patients with mild or no muscle dysfunction. Brain. 1996 Aug;119 ( Pt 4):1357-64. doi: 10.1093/brain/119.4.1357. PubMed PMID: 8813297.
Barrientos A, Casademont J, Nunes V. [Rearrangement of the mitochondrial DNA in Kearns-Sayre syndrome: is it necessary to differentiate deletions from duplications or polymerization?]. Neurologia. 1996 Aug-Sep;11(7):257-60. Review. PubMed PMID: 8974426.
Barrientos A, Casademont J, Rötig A, Miró O, Urbano-Márquez A, Rustin P, Cardellach F. Absence of relationship between the level of electron transport chain activities and aging in human skeletal muscle. Biochem Biophys Res Commun. 1996 Dec 13;229(2):536-9. doi: 10.1006/bbrc.1996.1839. PubMed PMID: 8954933.
Barrientos A, Casademont J, Genís D, Cardellach F, Fernández-Real JM, Grau JM, Urbano-Márquez A, Estivill X, 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. Hum Mutat. 1997;10(3):212-6. doi: 10.1002/(SICI)1098-1004(1997)10:3<212::AID-HUMU6>3.0.CO;2-K. PubMed PMID: 9298821.
Conget I, Barrientos A, Manzanares JM, Casademont J, Viñas O, Barceló J, Nunes V, Gomis R, Cardellach F. Respiratory chain activity and mitochondrial DNA content of nonpurified and purified pancreatic islet cells. Metabolism. 1997 Sep;46(9):984-7. doi: 10.1016/s0026-0495(97)90265-1. PubMed PMID: 9284883.
Barrientos A, Casademont J, Cardellach F, Ardite E, Estivill X, Urbano-Márquez A, Fernández-Checa JC, Nunes V. Qualitative and quantitative changes in skeletal muscle mtDNA and expression of mitochondrial-encoded genes in the human aging process. Biochem Mol Med. 1997 Dec;62(2):165-71. doi: 10.1006/bmme.1997.2647. PubMed PMID: 9441868.
Barrientos A, Casademont J, Cardellach F, Estivill X, Urbano-Marquez A, Nunes V. Reduced steady-state levels of mitochondrial RNA and increased mitochondrial DNA amount in human brain with aging. Brain Res Mol Brain Res. 1997 Dec 15;52(2):284-9. doi: 10.1016/s0169-328x(97)00278-7. PubMed PMID: 9495550.
Barrientos A, Moraes CT. Transmitochondrial microhybrids: a new system for the identification of nuclear genes controlling mitochondrial DNA maintenance and gene expression . In: Advances in Gene Technology: Molecular Biology in the Conquest of Disease. Ahmad F, editor. Miami: Oxford Univ. Press; 1998. . 65-66p.
Chretien D, Gallego J, Barrientos A, Casademont J, Cardellach F, Munnich A, Rötig A, Rustin P. Biochemical parameters for the diagnosis of mitochondrial respiratory chain deficiency in humans, and their lack of age-related changes. Biochem J. 1998 Jan 15;329 ( Pt 2):249-54. doi: 10.1042/bj3290249. PubMed PMID: 9425106; PubMed Central PMCID: PMC1219038.
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.
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.
Barrientos A, Kenyon L, Moraes CT. Human xenomitochondrial cybrids. Cellular models of mitochondrial complex I deficiency. J Biol Chem. 1998 Jun 5;273(23):14210-7. doi: 10.1074/jbc.273.23.14210. PubMed PMID: 9603924.
Masanés F, Barrientos A, Cebrián M, Pedrol E, Miró O, Casademont J, Grau JM. Clinical, histological and molecular reversibility of zidovudine myopathy. J Neurol Sci. 1998 Aug 14;159(2):226-8. doi: 10.1016/s0022-510x(98)00151-8. PubMed PMID: 9741412.
Barrientos A, Marín C, Miró O, Casademont J, Gómez M, Nunes V, Tolosa E, Urbano-Márquez A, Cardellach F. Biochemical and molecular effects of chronic haloperidol administration on brain and muscle mitochondria of rats. J Neurosci Res. 1998 Aug 15;53(4):475-81. doi: 10.1002/(SICI)1097-4547(19980815)53:4<475::AID-JNR9>3.0.CO;2-3. PubMed PMID: 9710267.
Barrientos A, Moraes CT. Simultaneous transfer of mitochondrial DNA and single chromosomes in somatic cells: a novel approach for the study of defects in nuclear-mitochondrial communication. Hum Mol Genet. 1998 Oct;7(11):1801-8. doi: 10.1093/hmg/7.11.1801. PubMed PMID: 9736783.
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.
Biarnés J, Barrientos A, Ricart W, Nunes V, Fernández-Castañer M, Soler J. [Diabetes mellitus associated with the A3243G mutation of mitochondrial DNA. Apropos a case]. Med Clin (Barc). 1999 Jan 30;112(3):99-101. Review. PubMed PMID: 10074618.
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.
Gómez 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]. Med Clin (Barc). 1999 Mar 13;112(9):326-9. PubMed PMID: 10220763.
Barrientos A, Moraes CT. Titrating the effects of mitochondrial complex I impairment in the cell physiology. J Biol Chem. 1999 Jun 4;274(23):16188-97. doi: 10.1074/jbc.274.23.16188. PubMed PMID: 10347173.
Jarreta D, Orús J, Barrientos A, Miró O, Roig E, Heras M, Moraes CT, Cardellach F, Casademont J. Mitochondrial function in heart muscle from patients with idiopathic dilated cardiomyopathy. Cardiovasc Res. 2000 Mar;45(4):860-5. doi: 10.1016/s0008-6363(99)00388-0. PubMed PMID: 10728411.
Mathur A, Hong Y, Kemp BK, Barrientos AA, Erusalimsky JD. Evaluation of fluorescent dyes for the detection of mitochondrial membrane potential changes in cultured cardiomyocytes. Cardiovasc Res. 2000 Apr;46(1):126-38. doi: 10.1016/s0008-6363(00)00002-x. PubMed PMID: 10727661.
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.
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.
Barrientos A, Müller S, Dey R, Wienberg J, Moraes CT. Cytochrome c oxidase assembly in primates is sensitive to small evolutionary variations in amino acid sequence. Mol Biol Evol. 2000 Oct;17(10):1508-19. doi: 10.1093/oxfordjournals.molbev.a026250. PubMed PMID: 11018157.
Dey R, Barrientos A, Moraes CT. Functional constraints of nuclear-mitochondrial DNA interactions in xenomitochondrial rodent cell lines. J Biol Chem. 2000 Oct 6;275(40):31520-7. doi: 10.1074/jbc.M004053200. PubMed PMID: 10908562.
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.
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.
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. J Biol Chem. 2001 Dec 14;276(50):46925-32. doi: 10.1074/jbc.M106429200. Epub 2001 Oct 10. PubMed PMID: 11595737.
Barrientos A, Casademont J, Rustin P, Cardellach F. Biochemical aspects of Ageing of skeletal muscle . In: Skeletal Muscle: Pathology and management of disease. Preedy V, Peters T, editors. London: Greenwich Medical Media Limited; 2002. . 85-95p.
Barrientos A, Korr D, Tzagoloff A. Shy1p is necessary for full expression of mitochondrial COX1 in the yeast model of Leigh's syndrome. EMBO J. 2002 Jan 15;21(1-2):43-52. doi: 10.1093/emboj/21.1.43. PubMed PMID: 11782424; PubMed Central PMCID: PMC125806.
Barrientos A, Barros MH, Valnot I, Rötig A, Rustin P, Tzagoloff A. Cytochrome oxidase in health and disease. Gene. 2002 Mar 6;286(1):53-63. doi: 10.1016/s0378-1119(01)00803-4. Review. PubMed PMID: 11943460.
Barrientos A. In vivo and in organello assessment of OXPHOS activities. Methods. 2002 Apr;26(4):307-16. doi: 10.1016/S1046-2023(02)00036-1. Review. PubMed PMID: 12054921.
Ludovico P, Rodrigues F, Almeida A, Silva MT, Barrientos A, Côrte-Real M. Cytochrome c release and mitochondria involvement in programmed cell death induced by acetic acid in Saccharomyces cerevisiae. Mol Biol Cell. 2002 Aug;13(8):2598-606. doi: 10.1091/mbc.e01-12-0161. PubMed PMID: 12181332; PubMed Central PMCID: PMC117928.
Barrientos A. Yeast models of human mitochondrial diseases. IUBMB Life. 2003 Feb;55(2):83-95. doi: 10.1002/tbmb.718540876. Review. PubMed PMID: 12749690.
Carlson CG, Barrientos A, Tzagoloff A, Glerum DM. COX16 encodes a novel protein required for the assembly of cytochrome oxidase in Saccharomyces cerevisiae. J Biol Chem. 2003 Feb 7;278(6):3770-5. doi: 10.1074/jbc.M209893200. Epub 2002 Nov 22. PubMed PMID: 12446688.
Barrientos A, Pierre D, Lee J, Tzagoloff A. Cytochrome oxidase assembly does not require catalytically active cytochrome C. J Biol Chem. 2003 Mar 14;278(11):8881-7. doi: 10.1074/jbc.M212427200. Epub 2003 Jan 8. PubMed PMID: 12522138.
Jin C, Barrientos A, Tzagoloff A. Yeast dihydroxybutanone phosphate synthase, an enzyme of the riboflavin biosynthetic pathway, has a second unrelated function in expression of mitochondrial respiration. J Biol Chem. 2003 Apr 25;278(17):14698-703. doi: 10.1074/jbc.M300593200. Epub 2003 Feb 20. PubMed PMID: 12595523.
Barrientos A, Korr D, Barwell KJ, Sjulsen C, Gajewski CD, Manfredi G, Ackerman S, Tzagoloff A. MTG1 codes for a conserved protein required for mitochondrial translation. Mol Biol Cell. 2003 Jun;14(6):2292-302. doi: 10.1091/mbc.e02-10-0636. Epub 2003 Apr 4. PubMed PMID: 12808030; PubMed Central PMCID: PMC194879.
Solans A, Zambrano A, Barrientos A. Cytochrome c oxidase deficiency: from yeast to human. Preclinica. 2004; 2(5):336-348.
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.
Barrientos A, Zambrano A, Tzagoloff A. Mss51p and Cox14p jointly regulate mitochondrial Cox1p expression in Saccharomyces cerevisiae. EMBO J. 2004 Sep 1;23(17):3472-82. doi: 10.1038/sj.emboj.7600358. Epub 2004 Aug 12. PubMed PMID: 15306853; PubMed Central PMCID: PMC516630.
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.
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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