Pyroptosis in inflammation-related respiratory disease

Y Feng, M Li, X Yangzhong, X Zhang, A Zu… - Journal of physiology …, 2022 - Springer
Pyroptosis is commonly induced by the gasdermin (GSDM) family and is accompanied by
the release of inflammatory cytokines such as IL-1β and IL-18. Recently, increasing …

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 …

[PDF][PDF] The impact of highly effective modulator therapy on cystic fibrosis microbiology and inflammation

LJ Caverly, SA Riquelme, KB Hisert - Clinics in chest medicine, 2022 - Elsevier
Highly-effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator
therapy (HEMT) corrects the underlying molecular defect causing cystic fibrosis (CF) …

[HTML][HTML] Recruited monocytes/macrophages drive pulmonary neutrophilic inflammation and irreversible lung tissue remodeling in cystic fibrosis

HH Öz, EC Cheng, C Di Pietro, T Tebaldi, G Biancon… - Cell reports, 2022 - cell.com
Persistent neutrophil-dominated lung inflammation contributes to lung damage in cystic
fibrosis (CF). However, the mechanisms that drive persistent lung neutrophilia and tissue …

Elexacaftor/tezacaftor/ivacaftor corrects monocyte microbicidal deficiency in cystic fibrosis

L Cavinato, FR Luly, V Pastore… - European …, 2023 - Eur Respiratory Soc
Background Cystic fibrosis (CF), which is caused by mutations in the CF transmembrane
conductance regulator (CFTR), is characterised by chronic bacterial lung infection and …

Comparative effects of CFTR modulators on phagocytic, metabolic and inflammatory profiles of CF and nonCF macrophages

DS Aridgides, DL Mellinger, LL Gwilt, TH Hampton… - Scientific Reports, 2023 - nature.com
Macrophage dysfunction has been well-described in Cystic Fibrosis (CF) and may contribute
to bacterial persistence in the lung. Whether CF macrophage dysfunction is related directly …

Post-approval studies with the CFTR modulators Elexacaftor-Tezacaftor—Ivacaftor

B Tümmler - Frontiers in Pharmacology, 2023 - frontiersin.org
Triple combination therapy with the CFTR modulators elexacaftor (ELX), tezacaftor (TEZ)
and ivacaftor (IVA) has been qualified as a game changer in cystic fibrosis (CF). We provide …

Impact of CFTR Modulators on the Impaired Function of Phagocytes in Cystic Fibrosis Lung Disease

A Meoli, O Eickmeier, G Pisi, V Fainardi… - International Journal of …, 2022 - mdpi.com
Cystic fibrosis (CF), the most common genetically inherited disease in Caucasian
populations, is a multi-systemic life-threatening autosomal recessive disorder caused by …

Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond

MA Mall, GJ Criner, M Miravitlles… - European …, 2023 - Eur Respiratory Soc
The cystic fibrosis transmembrane conductance regulator (CFTR) is a crucial ion channel for
transport of chloride and bicarbonate anions. Functional roles of CFTR have been identified …

[HTML][HTML] Body mass index and nutritional intake following Elexacaftor/Tezacaftor/Ivacaftor modulator therapy in adults with cystic fibrosis

LR Caley, HH Jarosz-Griffiths, L Smith, L Gale… - Journal of Cystic …, 2023 - Elsevier
Abstract Background Elexacaftor/Tezacaftor/Ivacaftor (ETI) modulator therapy is often
associated with increased body mass index (BMI) in people with cystic fibrosis (CF). This is …