The mucus clearance system is the dominant mechanical host defense system of the human
lung. Mucus is cleared from the lung by cilia and airflow, including both two-phase gas-liquid …

Cystic fibrosis (CF) is a lethal inherited disease caused by mutations in the CF
transmembrane conductance regulator (CFTR) gene, which result in impairment of CFTR …

Rationale: The CFTR (cystic fibrosis transmembrane conductance regulator) modulator
combination elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to improve clinical …

The availability of the human genome sequence and tools for interrogating individual
genomes provide an unprecedented opportunity to apply genetics to medicine. Mendelian …

Standard follow-up and symptomatic treatment have allowed most patients with cystic
fibrosis to live to young adulthood. However, many patients still die prematurely from …

RNA‐based therapeutics have the potential to revolutionize the treatment and prevention of
human diseases. While early research faced setbacks, it established the basis for …

Overlapping complementary DNA clones were isolated from epithelial cell libraries with a
genomic DNA segment containing a portion of the putative cystic fibrosis (CF) locus, which is …

Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in the gene encoding the
CF transmembrane conductance regulator (CFTR), a protein kinase A (PKA)-activated …

The cystic fibrosis transmembrane conductance regulator (CFTR) belongs to the ATP
binding cassette (ABC) transporter superfamily but functions as an anion channel crucial for …

Pulmonary defense mechanisms preventing chronic bacterial infection. The lung is depicted
as an inverted funnel, reflecting the relative surface area of distal versus proximal airways …