Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
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 …

CFTR modulators: the changing face of cystic fibrosis in the era of precision medicine

M Lopes-Pacheco - Frontiers in pharmacology, 2020 - frontiersin.org
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 …

Pharmacologic improvement of CFTR function rapidly decreases sputum pathogen density, but lung infections generally persist

DP Nichols, SJ Morgan, M Skalland… - The Journal of …, 2023 - Am Soc Clin Investig
Background Lung infections are among the most consequential manifestations of cystic
fibrosis (CF) and are associated with reduced lung function and shortened survival. Drugs …

Cystic fibrosis: emergence of highly effective targeted therapeutics and potential clinical implications

MA Mall, N Mayer-Hamblett… - American journal of …, 2020 - atsjournals.org
Cystic fibrosis (CF) remains the most common life-shortening hereditary disease in white
populations, with high morbidity and mortality related to chronic airway mucus obstruction …

CFTR modulator therapy: transforming the landscape of clinical care in cystic fibrosis

JL Taylor-Cousar, PD Robinson, M Shteinberg… - The Lancet, 2023 - thelancet.com
Following discovery of the cystic fibrosis transmembrane conductance regulator (CFTR)
gene in 1989 and subsequent elucidation of the varied CFTR protein abnormalities that …

Impact of elexacaftor–tezacaftor–ivacaftor on bacterial colonization and inflammatory responses in cystic fibrosis

S Sheikh, RD Britt Jr, NA Ryan‐Wenger… - Pediatric …, 2023 - Wiley Online Library
Background Cystic fibrosis (CF) is a multisystem disease with progressive deterioration.
Recently, CF transmembrane conductance regulator (CFTR) modulator therapies were …

Mucus, mucins, and cystic fibrosis

CB Morrison, MR Markovetz, C Ehre - Pediatric pulmonology, 2019 - Wiley Online Library
Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the
Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane …

Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy

KB Hisert, SE Birket, JP Clancy, DG Downey… - The Lancet …, 2023 - thelancet.com
Cystic fibrosis is a multiorgan disease caused by impaired function of the cystic fibrosis
transmembrane conductance regulator (CFTR). Since the introduction of the CFTR …

[HTML][HTML] PROMISE: working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy

DP Nichols, SH Donaldson, CA Frederick… - Journal of Cystic …, 2021 - Elsevier
Highly effective CFTR modulator drug therapy is increasingly available to those with cystic
fibrosis. Multiple observational research studies are now being conducted to better …

Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties via hydration

CB Morrison, KM Shaffer, KC Araba… - European …, 2022 - Eur Respiratory Soc
Question Cystic fibrosis (CF) is characterised by the accumulation of viscous adherent
mucus in the lungs. While several hypotheses invoke a direct relationship with cystic fibrosis …