The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis

Cystic fibrosis (CF) is a disease produced by mutations in the CFTR channel. We have previously reported that the CFTR chloride transport activity indirectly regulates the differential expression of several genes, including SRC and MUC1. Here we report that MT-ND4, a mitochondrial gene encoding a su...

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Autor principal: Valdivieso, A.G
Otros Autores: Marcucci, F., Taminelli, G., Guerrico, A.G, Álvarez, S., Teiber, M.L, Dankert, M.A, Santa-Coloma, T.A
Formato: Capítulo de libro
Lenguaje:Inglés
Publicado: 2007
Acceso en línea:Registro en Scopus
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Registro en la Biblioteca Digital
Aporte de:Registro referencial: Solicitar el recurso aquí
LEADER 09988caa a22011417a 4500
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024 7 |2 scopus  |a 2-s2.0-33947644153 
024 7 |2 cas  |a glibenclamide, 10238-21-8; reduced nicotinamide adenine dinucleotide dehydrogenase (ubiquinone), 9028-04-0; 3-((3-trifluoromethyl)phenyl)-5-((3-carboxyphenyl)methylene)-2-thioxo-4-thiazolidinone; Benzoic Acids; CFTR protein, human; Cystic Fibrosis Transmembrane Conductance Regulator, 126880-72-6; Glyburide, 10238-21-8; NADH Dehydrogenase, 1.6.99.3; NADH dehydrogenase subunit 4, 1.6.5.3; Thiazolidines 
040 |a Scopus  |b spa  |c AR-BaUEN  |d AR-BaUEN 
030 |a BBRCA 
100 1 |a Valdivieso, A.G. 
245 1 4 |a The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis 
260 |c 2007 
270 1 0 |m Santa-Coloma, T.A.; Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires (FCEyN, UBA)Argentina; email: tsantacoloma@gmail.com 
506 |2 openaire  |e Política editorial 
504 |a Riordan, J.R., Rommens, J.M., Kerem, B., Alon, N., Rozmahel, R., Grzelczak, Z., Zielenski, J., Tsui, L.-C., Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA (1989) Science, 245, pp. 1066-1073 
504 |a Liang, P., Pardee, A.B., Differential display of eukaryotic messenger RNA by means of the polymerase chain reaction (1992) Science, 257, pp. 967-971 
504 |a Gonzalez-Guerrico, A.M., Cafferata, E.G., Radrizzani, M., Marcucci, F., Gruenert, D., Pivetta, O.H., Favaloro, R.R., Santa-Coloma, T.A., Tyrosine kinase c-Src constitutes a bridge between cystic fibrosis transmembrane regulator channel failure and MUC1 overexpression in cystic fibrosis (2002) J. Biol. Chem., 277, pp. 17239-17247 
504 |a Lei, D.C., Kunzelmann, K., Koslowsky, T., Yezzi, M.J., Escobar, L.C., Xu, Z., Ellison, A.R., Gruenert, D.C., Episomal expression of wild-type CFTR corrects cAMP-dependent chloride transport in respiratory epithelial cells (1996) Gene Ther., 3, pp. 427-436 
504 |a Hirst, J., Carroll, J., Fearnley, I.M., Shannon, R.J., Walker, J.E., The nuclear encoded subunits of complex I from bovine heart mitochondria (2003) Biochim. Biophys. Acta., 1604, pp. 135-150 
504 |a Liang, P., Averboukh, L., Pardee, A.B., Distribution and cloning of eukaryotic mRNAs by means of differential display: refinements and optimization (1993) Nucleic Acids Res., 21, pp. 3269-3275 
504 |a Cafferata, E.G., Gonzalez-Guerrico, A.M., Pivetta, O.H., Santa-Coloma, T.A., Identification by differential display of a mRNA specifically induced by 12-O-tetradecanoylphorbol-13-acetate (TPA) in T84 human colon carcinoma cells (1996) Cell Mol. Biol. (Noisy-le-grand), 42, pp. 797-804 
504 |a Miller, F.J., Rosenfeldt, F.L., Zhang, C., Linnane, A.W., Nagley, P., Precise determination of mitochondrial DNA copy number in human skeletal and cardiac muscle by a PCR-based assay: lack of change of copy number with age (2003) Nucleic Acids Res., 31, pp. e61 
504 |a Ma, T., Thiagarajah, J.R., Yang, H., Sonawane, N.D., Folli, C., Galietta, L.J., Verkman, A.S., Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion (2002) J. Clin. Invest., 110, pp. 1651-1658 
504 |a Degli Esposti, M., Carelli, V., Ghelli, A., Ratta, M., Crimi, M., Sangiorgi, S., Montagna, P., Cortelli, P., Functional alterations of the mitochondrially encoded ND4 subunit associated with Leber's hereditary optic neuropathy (1994) FEBS Lett., 352, pp. 375-379 
504 |a Shapiro, B.L., Feigal, R.J., Lam, L.F., Mitochondrial NADH dehydrogenase in cystic fibrosis (1979) Proc. Natl. Acad. Sci .USA, 76, pp. 2979-2983 
504 |a Shapiro, B.L., Lam, L.F., Feigal, R.J., Mitochondrial NADH dehydrogenase in cystic fibrosis: enzyme kinetics in cultured fibroblasts (1982) Am. J. Hum. Genet., 34, pp. 846-852 
504 |a Dechecchi, M.C., Girella, E., Cabrini, G., Berton, G., The Km of NADH dehydrogenase is decreased in mitochondria of cystic fibrosis cells (1988) Enzyme, 40, pp. 45-50 
504 |a Shapiro, B.L., Evidence for a mitochondrial lesion in cystic fibrosis (1989) Life Sci., 44, pp. 1327-1334 
504 |a Shapiro, B.L., Mitochondrial dysfunction, energy expenditure, and cystic fibrosis (1988) Lancet, 2, p. 289 
504 |a Gattermann, N., Dadak, M., Hofhaus, G., Wulfert, M., Berneburg, M., Loeffler, M.L., Simmonds, H.A., Severe impairment of nucleotide synthesis through inhibition of mitochondrial respiration (2004) Nucleosides Nucleotides Nucleic Acids, 23, pp. 1275-1279 
504 |a de Meer, K., Jeneson, J.A., Gulmans, V.A., van der Laag, J., Berger, R., Efficiency of oxidative work performance of skeletal muscle in patients with cystic fibrosis (1995) Thorax, 50, pp. 980-983 
504 |a DiMauro, S., Exercise intolerance and the mitochondrial respiratory chain (1999) Ital J. Neurol. Sci., 20, pp. 387-393 
520 3 |a Cystic fibrosis (CF) is a disease produced by mutations in the CFTR channel. We have previously reported that the CFTR chloride transport activity indirectly regulates the differential expression of several genes, including SRC and MUC1. Here we report that MT-ND4, a mitochondrial gene encoding a subunit of the mitochondrial Complex I (mtCx-I), is also a CFTR-dependent gene. A reduced expression of MT-ND4 was observed in CFDE cells (derived from a CF patient) when compared to CFDE cells ectopically expressing wild-type CFTR. The differential expression of MT-ND4 in CF was confirmed by RT-PCR. In situ hybridizations of deparaffinized human lung tissue slices derived from wt-CFTR or CF patients also showed downregulation of ND4 in CF. In addition, the CFTR chloride transport inhibitors glibenclamide and CFTR(inh)-172 also reduced MT-ND4 expression in CFDE cells ectopically expressing wt CFTR. These results suggest that the CFTR chloride transport activity indirectly up-regulates MT-ND4 expression. © 2007 Elsevier Inc. All rights reserved.  |l eng 
536 |a Detalles de la financiación: National Science and Technology Development Agency, PICT 05-13970 
536 |a Detalles de la financiación: Universidad de Buenos Aires, X-138, X-156, TW67 
536 |a Detalles de la financiación: National Council for Scientific Research, PIP694/98 
536 |a Detalles de la financiación: The CFDE and CFDE/6RepCFTR cells were a gift from Dr. Dieter Gruenert. Human lung slices, from pathological archives, were generously provided by Dr. Guillermo Gallo, from the ``Hospital de Pediatría Prof. Dr. Juan P. Garrahan” and by Dr. Sergio V. Perrone, from the ``Fundación Favaloro”, Buenos Aires, Argentina. This work was supported by grants from the University of Buenos Aires (TW67, X-138, and X-156), the National Research Council of Argentina (PIP694/98), the National Agency for the Promotion of Science and Technology (PICT 05-13970), and the Ministry of Health of Argentina (Fellowship Carrillo-Oñativia, 2003–2004) to TASC. 
593 |a Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires (FCEyN, UBA), Argentina 
593 |a Instituto de Investigaciones Bioquímicas (IIB) de Buenos Aires, Consejo Nacional de Investigaciones Científicas y Técnicas (IIBBA, CONICET), IIB Fundación Campomar (now Fundación Instituto Leloir), Argentina 
593 |a Facultad de Ciencias Médicas, Programa de Investigaciones Biomédicas, Pontificia Universidad Católica Argentina, Buenos Aires, Argentina 
690 1 0 |a CFDE 
690 1 0 |a CFTR 
690 1 0 |a CFTR(INH)-172 
690 1 0 |a CYSTIC FIBROSIS 
690 1 0 |a GLIBENCLAMIDE 
690 1 0 |a MITOCHONDRIA 
690 1 0 |a MITOCHONDRIAL EXPRESSED GENES 
690 1 0 |a MT-ND4 
690 1 0 |a GLIBENCLAMIDE 
690 1 0 |a REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE) 
690 1 0 |a TRANSMEMBRANE CONDUCTANCE REGULATOR 
690 1 0 |a ARTICLE 
690 1 0 |a CONTROLLED STUDY 
690 1 0 |a CYSTIC FIBROSIS 
690 1 0 |a HISTOPATHOLOGY 
690 1 0 |a HUMAN 
690 1 0 |a HUMAN CELL 
690 1 0 |a HUMAN TISSUE 
690 1 0 |a IN SITU HYBRIDIZATION 
690 1 0 |a MITOCHONDRIAL GENE 
690 1 0 |a MT ND4 GENE 
690 1 0 |a NUCLEOTIDE SEQUENCE 
690 1 0 |a PRIORITY JOURNAL 
690 1 0 |a PROTEIN EXPRESSION 
690 1 0 |a BASE SEQUENCE 
690 1 0 |a BENZOIC ACIDS 
690 1 0 |a CELL LINE 
690 1 0 |a CYSTIC FIBROSIS 
690 1 0 |a CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR 
690 1 0 |a DOWN-REGULATION 
690 1 0 |a GENE EXPRESSION 
690 1 0 |a GLYBURIDE 
690 1 0 |a HUMANS 
690 1 0 |a IN SITU HYBRIDIZATION 
690 1 0 |a LUNG 
690 1 0 |a MOLECULAR SEQUENCE DATA 
690 1 0 |a NADH DEHYDROGENASE 
690 1 0 |a THIAZOLIDINES 
700 1 |a Marcucci, F. 
700 1 |a Taminelli, G. 
700 1 |a Guerrico, A.G. 
700 1 |a Álvarez, S. 
700 1 |a Teiber, M.L. 
700 1 |a Dankert, M.A. 
700 1 |a Santa-Coloma, T.A. 
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