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 t...

Descripción completa

Guardado en:
Detalles Bibliográficos
Autores principales: Valdivieso, Ángel Gabriel, Marcucci, Florencia, Taminelli, Guillermo, González Guerrico, Anatilde, Alvarez, Sergio, Dankert, Marcelo A., Teiber, María Luz, Santa Coloma, Tomás Antonio
Formato: Artículo
Lenguaje:Inglés
Inglés
Publicado: Elsevier 2019
Materias:
FIBROSIS QUISTICA
MITOCONDRIAS
GENES MITOCONDRIALES
GLIBURIDA
CFTR
MT-ND4
CFDE
CFTR(inh)-172
Acceso en línea:https://repositorio.uca.edu.ar/handle/123456789/1456
Aporte de:
Repositorio Institucional de la Universidad Católica Argentina (UCA) de Universidad Católica Argentina
id I33-R139123456789-1456
record_format dspace
institution Universidad Católica Argentina
institution_str I-33
repository_str R-139
collection Repositorio Institucional de la Universidad Católica Argentina (UCA)
language Inglés
Inglés
topic 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
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
topic_facet FIBROSIS QUISTICA
MITOCONDRIAS
GENES MITOCONDRIALES
GLIBURIDA
CFTR
MT-ND4
CFDE
CFTR(inh)-172
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
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 2019
url https://repositorio.uca.edu.ar/handle/123456789/1456
work_keys_str_mv AT valdiviesoangelgabriel theexpressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT marcucciflorencia theexpressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT taminelliguillermo theexpressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT gonzalezguerricoanatilde theexpressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT alvarezsergio theexpressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT dankertmarceloa theexpressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT teibermarialuz theexpressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT santacolomatomasantonio theexpressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT valdiviesoangelgabriel expressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT marcucciflorencia expressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT taminelliguillermo expressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT gonzalezguerricoanatilde expressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT alvarezsergio expressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT dankertmarceloa expressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT teibermarialuz expressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
AT santacolomatomasantonio expressionofthemitochondrialencodedgenend4isdownregulatedincysticfibrosis
bdutipo_str Repositorios
_version_ 1764820525556695041

Ejemplares similares