Your browser doesn't support javascript.
loading
Glued in lipids: Lipointoxication in cystic fibrosis.
Vandebrouck, Clarisse; Ferreira, Thierry.
Afiliação
  • Vandebrouck C; Laboratoire "Lipointoxication and Channelopathies (LiTch) - ConicMeds", Université de Poitiers, 1, rue Georges Bonnet, Poitiers, France; Laboratoire "Signalisation et Transports Ioniques Membranaires (STIM; EA 7349)", Université de Poitiers, 1, rue Georges Bonnet, Poitiers, France.
  • Ferreira T; Laboratoire "Lipointoxication and Channelopathies (LiTch) - ConicMeds", Université de Poitiers, 1, rue Georges Bonnet, Poitiers, France. Electronic address: thierry.ferreira@univ-poitiers.fr.
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.
Assuntos
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

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