Cystic fibrosis (CF) is a disease caused by mutations in the cystic fibrosis transmembrane
conductance regulator (CFTR) that in the airways result in reduced Cl− secretion and …

Primary airway epithelial cells grown in air-liquid interface differentiate into cultures that
resemble native epithelium morphologically, express ion transport similar to those in vivo …

The volume and composition of a thin layer of liquid covering the airway surface defend the
lung from inhaled pathogens and debris. Airway epithelia secrete Cl–into the airway surface …

In cystic fibrosis respiratory epithelial cells, the absence or dysfunction of the chloride
channel CFTR (Cystic Fibrosis Transmembrane conductance Regulator) results in reduced …

Pendrin, a Cl−/anion exchanger encoded by the gene PDS, is highly expressed in the
kidney, thyroid, and inner ear epithelia and is essential for bicarbonate secretion, iodide …

Key points Cl− and HCO3− had similar paracellular permeabilities in human airway
epithelia. PCl/PNa of airway epithelia was unaltered by pH 7.4 vs. pH 6.0 solutions. Under …

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene result in
defective epithelial cAMP-dependent Cl− secretion and increased airway Na+ absorption …

Cytoplasmic Ca 2+ is a master regulator of airway physiology; it controls fluid, mucus, and
antimicrobial peptide secretion, ciliary beating, and smooth muscle contraction. The focus of …

Objective/Hypothesis Ineffective mucociliary clearance (MCC) is a common
pathophysiologic process that underlies airway inflammation and infection. A dominant fluid …

Pendrin is an anion transporter encoded by the PDS/Pds gene. In humans, mutations in
PDS cause the genetic disorder Pendred syndrome, which is associated with deafness and …