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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Autores principales:	Valdivieso, A.G., Marcucci, F., Taminelli, G., Guerrico, A.G., Álvarez, S., Teiber, M.L., Dankert, M.A., Santa-Coloma, T.A.
Formato:	JOUR
Materias:	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
Acceso en línea:	http://hdl.handle.net/20.500.12110/paper_0006291X_v356_n3_p805_Valdivieso
Aporte de:	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) de Universidad de Buenos Aires

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spelling	todo:paper_0006291X_v356_n3_p805_Valdivieso2023-10-03T14:03:53Z The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis Valdivieso, A.G. Marcucci, F. Taminelli, G. Guerrico, A.G. Álvarez, S. Teiber, M.L. Dankert, M.A. Santa-Coloma, T.A. 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 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. Fil:Valdivieso, A.G. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Marcucci, F. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Taminelli, G. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Dankert, M.A. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Santa-Coloma, T.A. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. JOUR info:eu-repo/semantics/openAccess http://creativecommons.org/licenses/by/2.5/ar http://hdl.handle.net/20.500.12110/paper_0006291X_v356_n3_p805_Valdivieso
institution	Universidad de Buenos Aires
institution_str	I-28
repository_str	R-134
collection	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA)
topic	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
spellingShingle	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 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
topic_facet	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
description	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.
format	JOUR
author	Valdivieso, A.G. Marcucci, F. Taminelli, G. Guerrico, A.G. Álvarez, S. Teiber, M.L. Dankert, M.A. Santa-Coloma, T.A.
author_facet	Valdivieso, A.G. Marcucci, F. Taminelli, G. Guerrico, A.G. Álvarez, S. Teiber, M.L. Dankert, M.A. Santa-Coloma, T.A.
author_sort	Valdivieso, A.G.
title	The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis
title_short	The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis
title_full	The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis
title_fullStr	The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis
title_full_unstemmed	The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis
title_sort	expression of the mitochondrial gene mt-nd4 is downregulated in cystic fibrosis
url	http://hdl.handle.net/20.500.12110/paper_0006291X_v356_n3_p805_Valdivieso
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The expression of the mitochondrial gene MT-ND4 is downregulated in cystic fibrosis

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