The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function

Abstract: Cystic fibrosis (CF) is a frequent and lethal autosomal recessive disease. It results from different possible mutations in the CFTR gene, which encodes the CFTR chloride channel. We have previously studied the differential expression of genes in CF and CF corrected cell lines, and found a...

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Autores principales: Valdivieso, Ángel Gabriel, Clauzure, Mariángeles, Martín, María C., Taminelli, Guillermo, Massip Copiz, María Macarena, Sánchez, Francisco, Schulman, Gustavo, Teiber, María Luz, Santa Coloma, Tomás Antonio
Formato: Artículo
Lenguaje:Inglés
Publicado: Dominik Hartl, University of Tübingen, Germany 2019
Materias:
MEDICINA
FIBROSIS QUISTICA
GENES
CFTR
FUNCION MITOCONDRIAL
ENFERMEDADES
Acceso en línea:https://repositorio.uca.edu.ar/handle/123456789/8683
Aporte de:
Repositorio Institucional de la Universidad Católica Argentina (UCA) de Universidad Católica Argentina
id I33-R139123456789-8683
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
topic MEDICINA
FIBROSIS QUISTICA
GENES
CFTR
FUNCION MITOCONDRIAL
ENFERMEDADES
spellingShingle MEDICINA
FIBROSIS QUISTICA
GENES
CFTR
FUNCION MITOCONDRIAL
ENFERMEDADES
Valdivieso, Ángel Gabriel
Clauzure, Mariángeles
Martín, María C.
Taminelli, Guillermo
Massip Copiz, María Macarena
Sánchez, Francisco
Schulman, Gustavo
Teiber, María Luz
Santa Coloma, Tomás Antonio
The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function
topic_facet MEDICINA
FIBROSIS QUISTICA
GENES
CFTR
FUNCION MITOCONDRIAL
ENFERMEDADES
description Abstract: Cystic fibrosis (CF) is a frequent and lethal autosomal recessive disease. It results from different possible mutations in the CFTR gene, which encodes the CFTR chloride channel. We have previously studied the differential expression of genes in CF and CF corrected cell lines, and found a reduced expression of MTND4 in CF cells. MTND4 is a mitochondrial gene encoding the MTND4 subunit of the mitochondrial Complex I (mCx-I). Since this subunit is essential for the assembly and activity of mCx-I, we have now studied whether the activity of this complex was also affected in CF cells. By using Blue Native-PAGE, the in-gel activity (IGA) of the mCx-I was found reduced in CFDE and IB3-1 cells (CF cell lines) compared with CFDE/ 6RepCFTR and S9 cells, respectively (CFDE and IB3-1 cells ectopically expressing wild-type CFTR). Moreover, colon carcinoma T84 and Caco-2 cells, which express wt-CFTR, either treated with CFTR inhibitors (glibenclamide, CFTR(inh)-172 or GlyH101) or transfected with a CFTR-specific shRNAi, showed a significant reduction on the IGA of mCx-I. The reduction of the mCx-I activity caused by CFTR inhibition under physiological or pathological conditions may have a profound impact on mitochondrial functions of CF and non-CF cells.
format Artículo
author Valdivieso, Ángel Gabriel
Clauzure, Mariángeles
Martín, María C.
Taminelli, Guillermo
Massip Copiz, María Macarena
Sánchez, Francisco
Schulman, Gustavo
Teiber, María Luz
Santa Coloma, Tomás Antonio
author_facet Valdivieso, Ángel Gabriel
Clauzure, Mariángeles
Martín, María C.
Taminelli, Guillermo
Massip Copiz, María Macarena
Sánchez, Francisco
Schulman, Gustavo
Teiber, María Luz
Santa Coloma, Tomás Antonio
author_sort Valdivieso, Ángel Gabriel
title The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function
title_short The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function
title_full The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function
title_fullStr The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function
title_full_unstemmed The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function
title_sort mitochondrial complex i activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (cftr) function
publisher Dominik Hartl, University of Tübingen, Germany
publishDate 2019
url https://repositorio.uca.edu.ar/handle/123456789/8683
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