Fibrosis quística: patogenia bacteriana y moduladores del CFTR (regulador de conductancia transmembranal de la fibrosis quística).

Abstract

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.

• Authors: Silvia Y Vargas-Roldán, José L Lezana-Fernández, Jorge F Cerna-Cortés, Santiago Partida-Sánchez, José I Santos-Preciado, Roberto Rosales-Reyes
• Journal: Boletin medico del Hospital Infantil de Mexico (Bol Med Hosp Infant Mex) Vol. 79 Issue 4 Pg. 215-221 ( 2022) ISSN: 1665-1146 [Electronic] Mexico
• Vernacular Title: Cystic fibrosis: bacterial pathogenesis and CFTR (cystic fibrosis transmembrane conductance regulator) modulators.
• PMID: 36100204 (Publication Type: Journal Article, Review, Research Support, Non-U.S. Gov't)
• Copyright: Copyright: © 2022 Permanyer.

Chemical References

• CFTR protein, human
• Cystic Fibrosis Transmembrane Conductance Regulator

Topics

• Cystic Fibrosis
• Cystic Fibrosis Transmembrane Conductance Regulator (genetics, metabolism, therapeutic use)
• Epithelial Cells (metabolism, microbiology)
• Fibrosis
• Humans
• Pseudomonas aeruginosa