Capítulo de Libro
Mitochondrial Alterations and Oxidative Stress in Cystic Fibrosis
Título del libro: Oxidative Stress in Lung Diseases
Fecha de publicación:
2019
Editorial:
Springer
ISBN:
978-981-13-8412-7
Idioma:
Inglés
Clasificación temática:
Resumen
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.
Palabras clave:
OXIDATIVE-STRESS
,
LUNG-DISEASE
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Capítulos de libros(BIOMED)
Capítulos de libros de INSTITUTO DE INVESTIGACIONES BIOMEDICAS
Capítulos de libros de INSTITUTO DE INVESTIGACIONES BIOMEDICAS
Citación
Valdivieso, Ángel Gabriel; Mitochondrial Alterations and Oxidative Stress in Cystic Fibrosis; Springer; 1; 2019; 355-371
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