Lavage lipidomics signatures in children with cystic fibrosis and protracted bacterial bronchitis

Airway profile of bioactive lipids predicts early progression of lung disease in cystic fibrosis

“…Aside from these previous publications from our group, to our knowledge no other study explored the potential correlations in CF infant airways between structural lung disease and a comprehensive panel of bioactive lipids. A recent publication compared BALF lipids of CF children, children with protracted bacterial bronchitis, and healthy controls [26]. That study showed that total phospholipid species, the main components of surfactant, are not markedly different in CF, and confirmed that LPC was significantly higher in CF children compared to non-CF children, consistent with our previous and current study.…”

Distinctive lipid signatures of bronchial epithelial cells associated with cystic fibrosis drugs, including Trikafta

“…Quite surprisingly, compared with other "omes" (12,13), the lipidome of the human bronchial epithelium has been investigated less. Few papers describe analytical efforts in lipidomics directed toward bronchial epithelium (14), while most of the lipidomics work associated with CF research has been done on profiling and biomarker discovery in both plasma (15,16) and BAL fluid (17,18). At the cellular level, a successful CFTR rescue maneuver, beside promoting better folding and trafficking of CFTR, might be linked to significant changes in the overall lipid composition of the cell membranes, which could either favor or contrast the rescue itself.…”

Recombinant Acid Ceramidase Reduces Inflammation and Infection in Cystic Fibrosis

“…Reports have suggested that relative concentrations of ceramides may be important, with different chain lengths over- and underrepresented in the blood of people with CF and murine models ( 13 – 15 ). Lipidomic analysis of BAL fluid has recently demonstrated an altered profile in CF ( 16 , 17 ).…”