SLC26A4 encodes pendrin, a transporter exchanging anions such as chloride, bicarbonate,
and iodide. Loss of function mutations of SLC26A4 cause Pendred syndrome characterized …

Stimulation of the cystic fibrosis transmembrane conductance regulator (CFTR) by protease-
activated receptors (PARs) at the basolateral membranes and by adenosine receptors (ADO …

Defective epithelial chloride secretion occurs in humans with cystic fibrosis (CF), a genetic
defect due to loss of function of CFTR, a cAMP-activated chloride channel. In the airways …

Key points Raised arterial blood CO2 (hypercapnia) is a feature of many lung diseases. CO2
has been shown to act as a cell signalling molecule in human cells, notably by influencing …

Loss of cystic fibrosis transmembrane conductance regulator (CFTR) anion channel function
causes cystic fibrosis (CF) lung disease. CFTR is expressed in airway epithelia, but how CF …

Serous cells are the predominant site of cystic fibrosis transmembrane conductance
regulator expression in the airways, and they make a significant contribution to the volume …

Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR) Cl− channel, which mediates transepithelial Cl− transport in a variety of …

The cystic fibrosis transmembrane conductance regulator (CFTR) is a unique member of the
ATP-binding cassette (ABC) transporter superfamily that forms a Cl-channel with complex …

Background In cystic fibrosis (CF), loss of CF transmembrane conductance regulator (CFTR)-
dependent bicarbonate secretion precipitates the accumulation of viscous mucus in the …

Human airway serous cells secrete antibiotic-rich fluid, but, in cystic fibrosis (CF), Cl−-
dependent fluid secretion is impaired by defects in CF transmembrane conductance …