Introduction: Cystic fibrosis is an autosomal recessive disorder caused by mutations in the
cystic fibrosis transmembrane conductance regulator (CFTR) gene that codes for the CFTR …

Significant progress has been achieved in developing precision therapies for cystic fibrosis;
however, highly effective treatments that target the ion channel, CFTR, are not yet available …

Pulmonary infections with Pseudomonas aeruginosa and Burkholderia cepacia complex
(Bcc) are difficult to treat and related with high mortality in some diseases like cystic fibrosis …

Mucociliary clearance is a biomechanical mechanism of airway protection. It consists of the
active transport along the bronchial tree of the mucus, a fluid propelled by the coordinated …

The ability to regulate cell volume is crucial for normal physiology; equally the regulation of
extracellular fluid homeostasis is of great importance. Alteration of normal extracellular fluid …

In cystic fibrosis (CF) lungs, epithelial Na+ channel (ENaC) hyperactivity causes a reduction
in airway surface liquid volume, leading to decreased mucocilliary clearance, chronic …

Chronic obstructive pulmonary disease (COPD) and cystic fibrosis (CF) share molecular
mechanisms that cause the pathological symptoms they have in common. Here, we review …

Dysregulated induction of goblet cell differentiation results in excessive production and
retention of mucus and is a common feature of several chronic airways diseases. To date …

Induced pluripotent stem cells (iPSCs) are a recently developed technology in which fully
differentiated cells such as fibroblasts from individual CF patients can be repaired with …

The respiratory tract is lined with multiciliated epithelial cells that function to move mucus
and trapped particles via the mucociliary transport apparatus. Genetic and acquired …