Strong toll-like receptor responses in cystic fibrosis patients are associated with higher lung function.

ABSTRACT:

Background

Cystic fibrosis (CF) airways disease varies widely among patients with identical cystic fibrosis transmembrane conductance regulator (CFTR) genotypes. Robust airway inflammation is thought to be deleterious in CF; inter-individual variation in Toll-like receptor (TLR)-mediated innate immune inflammatory responses (TMIIR) might account for a portion of the phenotypic variation. We tested if TMIIR in people with CF are different than those of healthy controls, and whether higher TMIIR in people with CF are associated with reduced lung function.

Methods

We cultured whole blood from clinically stable subjects with CF (n = 76) and healthy controls (n = 45) with TLR agonists, and measured cytokine production and expression of TLR-associated genes. We tested for differen

SUBMITTER: Kosamo S 

PROVIDER: S-EPMC7272272 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature