Reduced Airway Surface pH Impairs Bacterial Killing in the Porcine Cystic Fibrosis Lung

ABSTRACT: Cystic fibrosis (CF) is a life-shortening disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Although bacterial lung infection and the resulting inflammation cause most of the morbidity and mortality, how loss of CFTR first disrupts airway host defense has remained uncertain. We asked what abnormality impairs elimination when a bacterium lands on the pristine surface of a newborn CF airway? To investigate this defect, we interrogated the viability of individual bacteria immobilized on solid grids and placed on the airway surface. As a model we studied CF pigs, which spontaneously develop hallmark features of CF lung disease. At birth, their lungs lack infection and inflammation, but have a reduced ability to eradicate bacteria. Here we

ORGANISM(S): Sus scrofa

SUBMITTER: Shyam Ramachandran 

PROVIDER: E-GEOD-36906 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress