Mitochondrial alterations and oxidative stress in cystic fibrosis

Abstract: Cystic fibrosis (CF) is the most frequent autosomal recessive disease and is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Since the discovery of the deletion in the phenylalanine 508 site (ΔF508) of the CFTR gene, the study of its function...

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Autor principal: Valdivieso, Ángel Gabriel
Formato: Parte de libro
Lenguaje:Inglés
Publicado: Springer Nature 2022
Materias:
FIBROSIS QUISTICA
ESTRES OXIDATIVO
CFRT
INMUNIDAD
Acceso en línea:https://repositorio.uca.edu.ar/handle/123456789/14243
Aporte de:
Repositorio Institucional de la Universidad Católica Argentina (UCA) de Universidad Católica Argentina
id I33-R139-123456789-14243
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 FIBROSIS QUISTICA
ESTRES OXIDATIVO
CFRT
INMUNIDAD
spellingShingle FIBROSIS QUISTICA
ESTRES OXIDATIVO
CFRT
INMUNIDAD
Valdivieso, Ángel Gabriel
Mitochondrial alterations and oxidative stress in cystic fibrosis
topic_facet FIBROSIS QUISTICA
ESTRES OXIDATIVO
CFRT
INMUNIDAD
description Abstract: Cystic fibrosis (CF) is the most frequent autosomal recessive disease and is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Since the discovery of the deletion in the phenylalanine 508 site (ΔF508) of the CFTR gene, the study of its function as chloride channel occupied most investigations. Now, we know that CFTR is also involved in the GSH and HCO3 − transport, and its function could regulate the mitochondrial function and ROS production. In this way, the notion of the CFTR as a simple chloride channel has begun to change toward a more complex function as molecular hub that integrates different cellular signals. There is a growing body of evidence that shows mitochondrial dysfunctions and increased oxidative stress in CF. Here, we review the mitochondrial defects induced by the altered function of the CFTR in CF, focusing on oxidative stress and inflammation as targets for therapy.
format Parte de libro
author Valdivieso, Ángel Gabriel
author_facet Valdivieso, Ángel Gabriel
author_sort Valdivieso, Ángel Gabriel
title Mitochondrial alterations and oxidative stress in cystic fibrosis
title_short Mitochondrial alterations and oxidative stress in cystic fibrosis
title_full Mitochondrial alterations and oxidative stress in cystic fibrosis
title_fullStr Mitochondrial alterations and oxidative stress in cystic fibrosis
title_full_unstemmed Mitochondrial alterations and oxidative stress in cystic fibrosis
title_sort mitochondrial alterations and oxidative stress in cystic fibrosis
publisher Springer Nature
publishDate 2022
url https://repositorio.uca.edu.ar/handle/123456789/14243
work_keys_str_mv AT valdiviesoangelgabriel mitochondrialalterationsandoxidativestressincysticfibrosis
bdutipo_str Repositorios
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