Global assessment of the integrated stress response in CF patient-derived airway and intestinal tissues.

ABSTRACT:

Background

Chronic inflammation is a hallmark among patients with cystic fibrosis (CF). We explored whether mutation-induced (F508del) misfolding of the cystic fibrosis transmembrane conductance regulator (CFTR), and/or secondary colonization with opportunistic pathogens, activate tissue remodeling and innate immune response drivers.

Methods

Using RNA-seq to interrogate global gene expression profiles, we analyzed stress response signaling cascades in primary human bronchial epithelia (HBE) and intestinal organoids.

Results

Primary HBE acquired from CF patients with advanced disease and prolonged exposure to pathogenic microorganisms display a clear molecular signature of activated tissue remodeling pathways, unfolded protein response (UPR), and chronic inflammation.

SUBMITTER: Bampi GB 

PROVIDER: S-EPMC7932027 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature