J Cyst Fibros 2022 May 4. Epub 2022 May 4.
Department of Biomedical Engineering, Columbia University, New York, NY, USA; Department of Medicine, Columbia University Irving Medical Center, New York, NY, USA; College of Dental Medicine, Columbia University Irving Medical Center, New York, NY, USA. Electronic address:
Background: Manifestations of cystic fibrosis, although well-characterized in the proximal airways, are understudied in the distal lung. Characterization of the cystic fibrosis lung 'matrisome' (matrix proteome) has not been previously described, and could help identify biomarkers and inform therapeutic strategies.
Methods: We performed liquid chromatography-mass spectrometry, gene ontology analysis, and multi-modal imaging, including histology, immunofluorescence, and electron microscopy for a comprehensive evaluation of distal human lung extracellular matrix (matrix) structure and composition in end-stage cystic fibrosis. Read More