Metabolomic responses to lumacaftor/ivacaftor in cystic fibrosis.

Benjamin T KoppScott McCullochChandra L ShresthaShuzhong ZhangLisa M SarzynskiFrederick W WoodleyDon Hayes

Published in: Pediatric pulmonology (2018)

Selected key lipid and amino acid metabolic pathways were significantly affected by Lumacaftor/Ivacaftor initiation and similar pathways were affected in clinical responders. Targeted metabolomics may provide useful and relevant biomarkers of CFTR modulator responses.

Keyphrases

cystic fibrosis
pseudomonas aeruginosa
amino acid
lung function
mass spectrometry
cancer therapy
fatty acid
drug delivery
chronic obstructive pulmonary disease