Ontology highlight
ABSTRACT:
SUBMITTER: Reihill JA
PROVIDER: S-EPMC8004921 | biostudies-literature | 2021 Mar
REPOSITORIES: biostudies-literature
Reihill James A JA Douglas Lisa E J LEJ Martin S Lorraine SL
Genes 20210322 3
Cystic fibrosis (CF) is a life-limiting genetic disorder caused by loss-of-function mutations in the gene which codes for the CF transmembrane conductance regulator (CFTR) Cl<sup>-</sup> channel. Loss of Cl<sup>-</sup> secretion across the apical membrane of airway lining epithelial cells results in dehydration of the airway surface liquid (ASL) layer which impairs mucociliary clearance (MCC), and as a consequence promotes bacterial infection and inflammation of the airways. Interventions that r ...[more]