Steve Bryson, PhD, science writer —

Small changes in the shape of CFTR, the protein defective or absent in people with cystic fibrosis (CF), opened its gate outside the cell to allow the flow of chloride ions, a study reported. CF-causing mutations that suppress CFTR function disrupt these long-distance shape changes. By comparison, CFTR potentiator…

Trikafta increased the production of the faulty CFTR protein in cystic fibrosis (CF) patients, but it remained in an immature state, a study suggests. These findings may shed light on the wide variability in response rates in CF patients and caution that immature CFTR protein may prevent sustained…

Exposure to traffic-related air pollution is an important predictor of early lung function decline in children and adolescents with cystic fibrosis (CF), a study has found. Community deprivation — reflecting poverty, housing, income, education, and health insurance coverage — was also a significant predictor of this decline in young…

Unique global changes in gene activity and protein production were linked to different types of mutations in the CFTR gene, the underlying cause of cystic fibrosis (CF), a cell-based study has revealed. These changes were most likely due to the cell’s attempt to cope with global gene activity changes…

Pulmonary exacerbations in adults with cystic fibrosis (CF) fall into three distinct groups based on inflammatory markers in their bloodstream, a pilot study has discovered. These unique inflammatory profiles corresponded to different times from the onset of symptoms to hospitalization, to the rate of lung function decline before…

Lipid levels in human saliva were found to be significantly linked to complications related to cystic fibrosis (CF) — such as poor lung function — and may act as a biomarker for assessing disease activity, according to a new study. These findings suggest that a non-invasive assessment of salivary…

CFTR modulators, approved therapies to treat cystic fibrosis (CF), showed antibacterial activity and enhanced the efficacy of some antibiotics against bacterial species commonly found in CF airways, according to a new study. “Additive effects or synergies were observed between the CFTR modulators and antibiotics against [two common] species” of…

Treatment with Trikafta improved insulin secretion and body weight within one year in people with cystic fibrosis (CF), according to a small analysis. Despite these findings, there were no consistent treatment-related improvements in measures relating to CF-related diabetes, or CFRD, a common complication among children and adults…

Researchers have discovered that monocytes, a type of white blood cell, drive persistent and sustained inflammation that leads to lung damage in people with cystic fibrosis (CF), a study reported. The researchers showed that, in a CF mouse model, reducing monocyte activity lowered the number of tissue-damaging immune cells…

Enrollment of adults at sites across the U.S. is continuing for a Phase 2a trial of AR-501 (gallium citrate), Aridis Pharmaceuticals‘ investigational inhaled therapy for chronic lung infections due to cystic fibrosis (CF). As an inhaled treatment, AR-501 is intended to be self-administered weekly using a hand-held nebulizer that…