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

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

Abstract: Cystic fibrosis (CF) is a disease produced by mutations in the CFTR channel. We have previously reported that the CFTR chloride transport activity regulates the differential expression of several genes, including SRC. 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 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, glibenclamide or CFTR(inh)-172 (CFTR chloride transport inhibitors) reduced MT-ND4 expression in cells expressing wt CFTR. These results suggest that the CFTR chloride transport activity indirectly up-regulates MT-ND4 expression.

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Main Authors: Valdivieso, Ángel Gabriel, Marcucci, Florencia, Taminelli, Guillermo, González Guerrico, Anatilde, Alvarez, Sergio, Dankert, Marcelo A., Teiber, María Luz, Santa Coloma, Tomás Antonio
Format: Artículo biblioteca
Language:eng
eng
Published: Elsevier 2007
Subjects:FIBROSIS QUISTICA, MITOCONDRIAS, GENES MITOCONDRIALES, GLIBURIDA, CFTR, MT-ND4, CFDE, CFTR(inh)-172,
Online Access:https://repositorio.uca.edu.ar/handle/123456789/1456
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spelling oai:ucacris:123456789-14562021-05-05T23:35:45Z The expression of the mitochondrial encoded gene ND4 is downregulated in cystic fibrosis Valdivieso, Ángel Gabriel Marcucci, Florencia Taminelli, Guillermo González Guerrico, Anatilde Alvarez, Sergio Dankert, Marcelo A. Teiber, María Luz Santa Coloma, Tomás Antonio FIBROSIS QUISTICA MITOCONDRIAS GENES MITOCONDRIALES GLIBURIDA CFTR MT-ND4 CFDE CFTR(inh)-172 Abstract: Cystic fibrosis (CF) is a disease produced by mutations in the CFTR channel. We have previously reported that the CFTR chloride transport activity regulates the differential expression of several genes, including SRC. 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 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, glibenclamide or CFTR(inh)-172 (CFTR chloride transport inhibitors) reduced MT-ND4 expression in cells expressing wt CFTR. These results suggest that the CFTR chloride transport activity indirectly up-regulates MT-ND4 expression. 2019-05-02T13:56:18Z 2019-05-02T13:56:18Z 2007 Artículo Valdivieso, A. G., Marcucci, F., Taminelli, G., González Guerrico, A., Álvarez, S., Teiber, M. L., Dankert, M. A., Santa-Coloma, T. A. The expression of the mitochondrial encoded gene ND4 is downregulated in cystic fibrosis. Biochemical and Biophysical Research Communications. 2007, 356 (3). doi:10.1016/j.bbrc.2007.03.057. Disponible en: https://repositorio.uca.edu.ar/handle/123456789/1456 0006-291X https://repositorio.uca.edu.ar/handle/123456789/1456 10.1016/j.bbrc.2007.03.057 17382898 eng eng Acceso Abierto https://creativecommons.org/licenses/by-nc-sa/4.0/ application/pdf Elsevier Biochemical and Biophysical Research Communications. 2007, 356 (3)
institution UCA
collection DSpace
country Argentina
countrycode AR
component Bibliográfico
access En linea
databasecode dig-uca
tag biblioteca
region America del Sur
libraryname Sistema de bibliotecas de la UCA
language eng
eng
topic FIBROSIS QUISTICA
MITOCONDRIAS
GENES MITOCONDRIALES
GLIBURIDA
CFTR
MT-ND4
CFDE
CFTR(inh)-172
FIBROSIS QUISTICA
MITOCONDRIAS
GENES MITOCONDRIALES
GLIBURIDA
CFTR
MT-ND4
CFDE
CFTR(inh)-172
spellingShingle FIBROSIS QUISTICA
MITOCONDRIAS
GENES MITOCONDRIALES
GLIBURIDA
CFTR
MT-ND4
CFDE
CFTR(inh)-172
FIBROSIS QUISTICA
MITOCONDRIAS
GENES MITOCONDRIALES
GLIBURIDA
CFTR
MT-ND4
CFDE
CFTR(inh)-172
Valdivieso, Ángel Gabriel
Marcucci, Florencia
Taminelli, Guillermo
González Guerrico, Anatilde
Alvarez, Sergio
Dankert, Marcelo A.
Teiber, María Luz
Santa Coloma, Tomás Antonio
The expression of the mitochondrial encoded gene ND4 is downregulated in cystic fibrosis
description Abstract: Cystic fibrosis (CF) is a disease produced by mutations in the CFTR channel. We have previously reported that the CFTR chloride transport activity regulates the differential expression of several genes, including SRC. 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 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, glibenclamide or CFTR(inh)-172 (CFTR chloride transport inhibitors) reduced MT-ND4 expression in cells expressing wt CFTR. These results suggest that the CFTR chloride transport activity indirectly up-regulates MT-ND4 expression.
format Artículo
topic_facet FIBROSIS QUISTICA
MITOCONDRIAS
GENES MITOCONDRIALES
GLIBURIDA
CFTR
MT-ND4
CFDE
CFTR(inh)-172
author Valdivieso, Ángel Gabriel
Marcucci, Florencia
Taminelli, Guillermo
González Guerrico, Anatilde
Alvarez, Sergio
Dankert, Marcelo A.
Teiber, María Luz
Santa Coloma, Tomás Antonio
author_facet Valdivieso, Ángel Gabriel
Marcucci, Florencia
Taminelli, Guillermo
González Guerrico, Anatilde
Alvarez, Sergio
Dankert, Marcelo A.
Teiber, María Luz
Santa Coloma, Tomás Antonio
author_sort Valdivieso, Ángel Gabriel
title The expression of the mitochondrial encoded gene ND4 is downregulated in cystic fibrosis
title_short The expression of the mitochondrial encoded gene ND4 is downregulated in cystic fibrosis
title_full The expression of the mitochondrial encoded gene ND4 is downregulated in cystic fibrosis
title_fullStr The expression of the mitochondrial encoded gene ND4 is downregulated in cystic fibrosis
title_full_unstemmed The expression of the mitochondrial encoded gene ND4 is downregulated in cystic fibrosis
title_sort expression of the mitochondrial encoded gene nd4 is downregulated in cystic fibrosis
publisher Elsevier
publishDate 2007
url https://repositorio.uca.edu.ar/handle/123456789/1456
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