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

Cystic Fibrosis pulmonary disease is characterized by chronic airway infections and concomitant non-resolving inflammation that usually leads to respiratory failure within the fourth decade of life. The disease results from patients bearing two mutant copies of the cystic fibrosis transmembrane conductance regulator (CFTR), a channel that transports chloride and bicarbonate [1]. Over 2000 different mutations in CFTR have been identified, and these mutations are associated with varying severity of disease based on how much residual CFTR activity remains.

Source: Journal of Cystic Fibrosis - December 4, 2019 Category: Respiratory Medicine Authors: Susanna Kosamo, Katherine B. Hisert, Victoria Dmyterko, Catherine Nguyen, R. Anthony Black, Tarah D. Holden, Frank Radella, Pauline A. Cotten, Christopher H. Goss, Moira L. Aitken, Mark M. Wurfel Tags: Original Article Source Type: research