TY - JOUR
T1 - Fibrosis quística
T2 - patogenia bacteriana y moduladores del CFTR (regulador de conductancia transmembranal de la fibrosis quística)
AU - Vargas-Roldán, Silvia Y.
AU - Lezana-Fernández, José L.
AU - Cerna-Cortés, Jorge F.
AU - Partida-Sánchez, Santiago
AU - Santos-Preciado, José I.
AU - Rosales-Reyes, Roberto
N1 - Publisher Copyright:
© 2021 Hospital Infantil de México Federico Gómez. Publicado por Permanyer.
PY - 2022/7/1
Y1 - 2022/7/1
N2 - 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.
AB - 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.
KW - Burkholderia cenocepacia
KW - CFTR modulators
KW - Cystic fibrosis
KW - Pseudomonas aeruginosa
UR - http://www.scopus.com/inward/record.url?scp=85137769138&partnerID=8YFLogxK
U2 - 10.24875/BMHIM.21000128
DO - 10.24875/BMHIM.21000128
M3 - Artículo
C2 - 36100204
AN - SCOPUS:85137769138
SN - 0539-6115
VL - 79
SP - 215
EP - 221
JO - Boletin Medico del Hospital Infantil de Mexico
JF - Boletin Medico del Hospital Infantil de Mexico
IS - 4
ER -