Glued in lipids: Lipointoxication in cystic fibrosis.
EBioMedicine
; 61: 103038, 2020 Nov.
Article
em En
| MEDLINE
| ID: mdl-33038767
ABSTRACT
Cystic Fibrosis (CF) is an autosomal recessive disease caused by mutations in the CF transmembrane regulator (CFTR) gene, which encodes a chloride channel located at the apical surface of epithelial cells. Unsaturated Fatty Acid (UFA) deficiency has been a persistent observation in tissues from patients with CF. However, the impacts of such deficiencies on the etiology of the disease have been the object of intense debates. The aim of the present review is first to highlight the general consensus on fatty acid dysregulations that emerges from, sometimes apparently contradictory, studies. In a second step, a unifying mechanism for the potential impacts of these fatty acid dysregulations in CF cells, based on alterations of membrane biophysical properties (known as lipointoxication), is proposed. Finally, the contribution of lipointoxication to the progression of the CF disease and how it could affect the efficacy of current treatments is also discussed.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Fibrose Cística
/
Metabolismo dos Lipídeos
Tipo de estudo:
Etiology_studies
Limite:
Humans
Idioma:
En
Revista:
EBioMedicine
Ano de publicação:
2020
Tipo de documento:
Article
País de afiliação:
França