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Título : The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function
Autor : 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 
Fecha de publicación : 2012
Editorial : Dominik Hartl, University of Tübingen, Germany
Cita : Valdivieso AG, Clauzure M, Marı´n MC, Taminelli GL, Massip Copiz MM, et al. (2012) The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function. PLoS ONE 7(11): e48059. doi:10.1371/journal.pone.0048059. Disponible en:
Resumen : 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.
ISSN : 1932-6203 (electrónico)
Disciplina: MEDICINA
DOI: doi:10.1371/journal.pone.0048059
Derechos: Acceso abierto
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