{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Laverty SL"],"funding":["Cystic Fibrosis Trust"],"pagination":["106131"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC12878693"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["124"],"pubmed_abstract":["<h4>Background</h4>The impact of CFTR modulator therapy on host immunity and outcomes in people with Cystic Fibrosis (CF)-related Aspergillus lung disease is poorly defined. We aimed to characterise fungal-relevant clinical outcomes post-CFTR modulators and assess effects on the Aspergillus-dependent Type I/III interferome.<h4>Methods</h4>Biomarkers of Aspergillus-related lung disease (Aspergillus-specific IgE/IgG), anti-fungal and corticosteroid therapy were analysed in a retrospective cohort of people with CF pre and post Elexacaftor/Tezacaftor/Ivacaftor (ETI) modulator therapy. Homozygous F508del (CF) and CFTR TALEN-corrected bronchial epithelial cells (BECs) were challenged with Aspergillus conidia and hyphae in the presence or absence of ETI CFTR modulator therapy with bulk RNA transc"],"journal":["EBioMedicine"],"pubmed_title":["CFTR modulators partially restore the epithelial interferome in Aspergillus infection to improve clinical outcome."],"pmcid":["PMC12878693"],"funding_grant_id":["SRC015"],"pubmed_authors":["Hopwood B","Shah A","Felton I","Laverty SL","Armstrong-James D","Kelleher P","Casey M","Simmonds N"],"additional_accession":[]},"is_claimable":false,"name":"CFTR modulators partially restore the epithelial interferome in Aspergillus infection to improve clinical outcome.","description":"<h4>Background</h4>The impact of CFTR modulator therapy on host immunity and outcomes in people with Cystic Fibrosis (CF)-related Aspergillus lung disease is poorly defined. We aimed to characterise fungal-relevant clinical outcomes post-CFTR modulators and assess effects on the Aspergillus-dependent Type I/III interferome.<h4>Methods</h4>Biomarkers of Aspergillus-related lung disease (Aspergillus-specific IgE/IgG), anti-fungal and corticosteroid therapy were analysed in a retrospective cohort of people with CF pre and post Elexacaftor/Tezacaftor/Ivacaftor (ETI) modulator therapy. Homozygous F508del (CF) and CFTR TALEN-corrected bronchial epithelial cells (BECs) were challenged with Aspergillus conidia and hyphae in the presence or absence of ETI CFTR modulator therapy with bulk RNA transc","dates":{"release":"2026-01-01T00:00:00Z","publication":"2026 Feb","modification":"2026-07-15T13:15:48.449Z","creation":"2026-07-05T03:08:11.693Z"},"accession":"S-EPMC12878693","cross_references":{"pubmed":["41621406"],"doi":["10.1016/j.ebiom.2026.106131"]}}