Pathological remodeling of distal lung matrix in end-stage cystic fibrosis patients.

ABSTRACT:

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.

Results

Quantitative proteomic profiling identified sixty-eight (68) matrix constituents with significantly altered expression in end-stage cystic f

SUBMITTER: Pinezich MR 

PROVIDER: S-EPMC10050894 | biostudies-literature | 2022 Nov

REPOSITORIES: biostudies-literature