Author: Perra, Léa; Balloy, Viviane; Foussignière, Tobias; Moissenet, Didier; Petat, Hortense; Mungrue, Imran N.; Touqui, Lhousseine; Corvol, Harriet; Chignard, Michel; Guillot, Loic
Title: CHAC1 Is Differentially Expressed in Normal and Cystic Fibrosis Bronchial Epithelial Cells and Regulates the Inflammatory Response Induced by Pseudomonas aeruginosa Document date: 2018_11_29
ID: 10fe70kl_2
Snippet: Keywords: cystic fibrosis, bronchial epithelium, Pseudomonas aeruginosa, inflammation, ChaC glutathionespecific γ-glutamylcyclotransferase 1, ER stress INTRODUCTION Cystic fibrosis (CF), the most common severe autosomal recessive genetic disease in the Caucasian population, is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene (1) (2) (3) . Lung injury is predominant and is the leading cause of morbidity and mortality .....
Document: Keywords: cystic fibrosis, bronchial epithelium, Pseudomonas aeruginosa, inflammation, ChaC glutathionespecific γ-glutamylcyclotransferase 1, ER stress INTRODUCTION Cystic fibrosis (CF), the most common severe autosomal recessive genetic disease in the Caucasian population, is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene (1) (2) (3) . Lung injury is predominant and is the leading cause of morbidity and mortality in CF patients. In airways, CFTR dysfunction leads to alterations in mucociliary clearance and impairment of innate immune host defenses. This results in chronic bacterial infection and inflammation, leading to degradation of the lung epithelium and progressive lung damage in CF patients (4) . Pseudomonas aeruginosa (Pa) is the leading cause of chronic pulmonary infection in the airways of adults with CF (5) . Pa colonization induces a strong inflammatory response in CF patients, particularly neutrophil accumulation and hypersecretion of inflammatory cytokines, such as IL-8 (6, 7) .
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