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Valdivieso, A.G.; Marcucci, F.; Taminelli, G.; Guerrico, A.G.; Álvarez, S.; Teiber, M.L.; Dankert, M.A.; Santa-Coloma, T.A. "The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis" (2007) Biochemical and Biophysical Research Communications. 356(3):805-809
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Abstract:

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.

Registro:

Documento: Artículo
Título:The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis
Autor:Valdivieso, A.G.; Marcucci, F.; Taminelli, G.; Guerrico, A.G.; Álvarez, S.; Teiber, M.L.; Dankert, M.A.; Santa-Coloma, T.A.
Filiación:Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires (FCEyN, UBA), Argentina
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
Facultad de Ciencias Médicas, Programa de Investigaciones Biomédicas, Pontificia Universidad Católica Argentina, Buenos Aires, Argentina
Palabras clave:CFDE; CFTR; CFTR(inh)-172; Cystic fibrosis; Glibenclamide; Mitochondria; Mitochondrial expressed genes; MT-ND4; glibenclamide; reduced nicotinamide adenine dinucleotide dehydrogenase (ubiquinone); transmembrane conductance regulator; article; controlled study; cystic fibrosis; histopathology; human; human cell; human tissue; in situ hybridization; mitochondrial gene; mt nd4 gene; nucleotide sequence; priority journal; protein expression; Base Sequence; Benzoic Acids; Cell Line; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Down-Regulation; Gene Expression; Glyburide; Humans; In Situ Hybridization; Lung; Molecular Sequence Data; NADH Dehydrogenase; Thiazolidines
Año:2007
Volumen:356
Número:3
Página de inicio:805
Página de fin:809
DOI: http://dx.doi.org/10.1016/j.bbrc.2007.03.057
Título revista:Biochemical and Biophysical Research Communications
Título revista abreviado:Biochem. Biophys. Res. Commun.
ISSN:0006291X
CODEN:BBRCA
CAS: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
Registro:https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_0006291X_v356_n3_p805_Valdivieso

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Citas:

---------- APA ----------
Valdivieso, A.G., Marcucci, F., Taminelli, G., Guerrico, A.G., Álvarez, S., Teiber, M.L., Dankert, M.A.,..., Santa-Coloma, T.A. (2007) . The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis. Biochemical and Biophysical Research Communications, 356(3), 805-809.
http://dx.doi.org/10.1016/j.bbrc.2007.03.057
---------- CHICAGO ----------
Valdivieso, A.G., Marcucci, F., Taminelli, G., Guerrico, A.G., Álvarez, S., Teiber, M.L., et al. "The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis" . Biochemical and Biophysical Research Communications 356, no. 3 (2007) : 805-809.
http://dx.doi.org/10.1016/j.bbrc.2007.03.057
---------- MLA ----------
Valdivieso, A.G., Marcucci, F., Taminelli, G., Guerrico, A.G., Álvarez, S., Teiber, M.L., et al. "The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis" . Biochemical and Biophysical Research Communications, vol. 356, no. 3, 2007, pp. 805-809.
http://dx.doi.org/10.1016/j.bbrc.2007.03.057
---------- VANCOUVER ----------
Valdivieso, A.G., Marcucci, F., Taminelli, G., Guerrico, A.G., Álvarez, S., Teiber, M.L., et al. The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis. Biochem. Biophys. Res. Commun. 2007;356(3):805-809.
http://dx.doi.org/10.1016/j.bbrc.2007.03.057