Abstract

VARGAS-ROLDAN, Silvia Y. et al. Cystic fibrosis: bacterial pathogenesis and CFTR (cystic fibrosis transmembrane conductance regulator) modulators. Bol. Med. Hosp. Infant. Mex. [online]. 2022, vol.79, n.4, pp.215-221.  Epub Sep 28, 2022. ISSN 1665-1146.  https://doi.org/10.24875/bmhim.21000128.

Cystic fibrosis is an autosomal recessive inherited disease caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). CFTR is a protein that transports ions across the membrane of lung epithelial cells. Loss of its function leads to the production of thick sticky mucus, where various bacterial pathogens can establish and adapt, contributing to the gradual loss of lung function. In this review, evidence of the molecular mechanisms used by Pseudomonas aeruginosa and Burkholderia cenocepacia to survive and persist in the pulmonary environment will be provided. Additionally, new therapeutic strategies based on CFTR function modulators will be described.

Keywords : Cystic fibrosis; Pseudomonas aeruginosa; Burkholderia cenocepacia; CFTR modulators.