Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

• Published in: Nature (London) Vol. 487; no. 7405; pp. 109 - 113
• Main Authors: PEZZULO, Alejandro A, XIAO XIAO TANG, BANFI, Botond, HORSWILL, Alexander R, STOLTZ, David A, MCCRAY, Paul B, WELSH, Michael J, ZABNER, Joseph, HOEGGER, Mark J, ABOU ALAIWA, Mahmoud H, RAMACHANDRAN, Shyam, MONINGER, Thomas O, KARP, Phillip H, WOHLFORD-LENANE, Christine L, HAAGSMAN, Henk P, VAN EIJK, Martin
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group 05.07.2012
• Subjects: Airway (Medicine); Animals; Animals, Newborn; Anti-Infective Agents - pharmacology; Antimicrobial agents; Bacteria; Bicarbonates - metabolism; Biological and medical sciences; Body Fluids - drug effects; Body Fluids - metabolism; Cystic fibrosis; Cystic Fibrosis - metabolism; Cystic Fibrosis - microbiology; Cystic Fibrosis - pathology; Cystic Fibrosis - therapy; Cystic Fibrosis Transmembrane Conductance Regulator - genetics; Cystic Fibrosis Transmembrane Conductance Regulator - metabolism; Development and progression; Disease; Disease Models, Animal; Errors of metabolism; Female; Health aspects; Hydrogen-ion concentration; Hydrogen-Ion Concentration - drug effects; Ion Transport; Lung - metabolism; Lung - microbiology; Lung - pathology; Lungs; Male; Medical sciences; Metabolic diseases; Microbial Viability - drug effects; Miscellaneous hereditary metabolic disorders; Mortality; Physiological aspects; Respiratory System - metabolism; Staphylococcus infections; Studies; Sus scrofa - microbiology; Swine; United States; Biological fluid; Respiratory disease; Metabolic diseases; Cystic fibrosis; Genetic disease; Bactericidal effect; Infection; Respiratory tract; Vertebrata; Mammalia; Swine; Newborn animal; Bacteriosis; pH; Digestive diseases; Artiodactyla; Ungulata; Pancreatic disease

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 the loss of CFTR function first disrupts airwa...