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 …

Modulator-refractory cystic fibrosis: Defining the scope and challenges of an emerging at-risk population

L Somerville, L Borish, I Noth… - Therapeutic Advances in …, 2024 - journals.sagepub.com
Cystic fibrosis (CF) causes life-shortening respiratory and systemic disease due to
dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride …

Inhibiting CFTR through inh-172 in primary neutrophils reveals CFTR-specific functional defects

AL Da Silva Cunha, M Blanter, J Renders, M Gouwy… - Scientific Reports, 2024 - nature.com
The lungs of people with cystic fibrosis (PwCF) are characterized by recurrent bacterial
infections and inflammation. Infections in cystic fibrosis (CF) are left unresolved despite …

Cystic fibrosis in the era of CFTR modulators: did the neutrophil slip through the cracks?

V Witko-Sarsat, PR Burgel - Journal of Leukocyte Biology, 2024 - academic.oup.com
Cystic fibrosis in the era of CFTR modulators: did the neutrophil slip through the cracks? Page
1 Cystic fibrosis in the era of CFTR modulators: did the neutrophil slip through the cracks …

Flow cytometric measurement of CFTR-mediated chloride transport in human neutrophils

A Montanez-Barragan, FH Robledo-Avila… - Journal of Leukocyte …, 2025 - academic.oup.com
Immune cells express a variety of ion channels and transporters in the plasma membrane
and intracellular organelles, responsible of the transference of charged ions across …

Anti-inflammatory effects of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis heterozygous for F508del

HH Jarosz-Griffiths, L Gillgrass, LR Caley, G Spoletini… - Plos one, 2024 - journals.plos.org
Inflammation is a key driver in the pathogenesis of cystic fibrosis (CF). We assessed the
effectiveness of elexacaftor/tezacaftor/ivacaftor (ETI) therapy on downregulating systemic …

[PDF][PDF] Pneumonia and parapneumonic pleural effusions in adults with Cystic Fibrosis

G Spoletini, H Saumtaully, AP Sawant… - ERJ Open …, 2025 - publications.ersnet.org
Extract Cystic fibrosis (CF) is caused by an absence or dysfunction of the transmembrane
conductance regulator (CFTR) protein, an anion channel exchanging chloride and …

[PDF][PDF] Virulence mechanisms of the cystic fibrosis pathogen Achromobacter xylosoxidans

C Sahl - 2024 - lup.lub.lu.se
Cystic fibrosis (CF) is a genetic disorder caused by mutations in the chloride channel cystic
fibrosis transmembrane conductance regulator (CFTR). The defective ion transport causes …