Cystic Fibrosis Transmembrane Conductance Regulator: Roles in Chronic Obstructive Pulmonary Disease.

Dransfield, Mark, Rowe, Steven ORCID: 0000-0001-9045-0133, Vogelmeier, Claus F ORCID: 0000-0002-9798-2527, Wedzicha, Jadwiga, Criner, Gerard J, Han, MeiLan K, Martinez, Fernando J and Calverley, Peter ORCID: 0000-0003-4676-9993 (2022) Cystic Fibrosis Transmembrane Conductance Regulator: Roles in Chronic Obstructive Pulmonary Disease. American journal of respiratory and critical care medicine, 205 (6). pp. 631-640.

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Official URL: http://dx.doi.org/10.1164/rccm.202109-2064tr

Abstract

Chronic obstructive pulmonary disease (COPD) manifests with a variety of clinical presentations, reflecting its complex pathology. Currently, care focuses on symptom amelioration and prevention of complications and thus is generally tailored to disease severity rather than targeting specific pathophysiologic mechanisms. Chronic inflammation and mucus hypersecretion are key features of COPD. Epithelial ion channel dysfunction may be important, as it results in airway dehydration and defective host defense, contributing to chronic airway inflammation. Recent evidence suggests considerable similarities between COPD and cystic fibrosis (CF), a disease in which chloride ion channel dysfunction has been extensively studied (in particular CFTR [CF transmembrane conductance regulator]). Understanding commonalities between CF and COPD, and the role of CFTR in CF, may help in designing strategies targeting ion channel dysfunction and lead to new treatments with potential to alter the natural history of disease progression. Here, we review the roles of airway mucus and CFTR in normal lung function, the previously underestimated contribution of mucus stasis to the development of COPD, and the evidence for targeting CFTR to counteract mucus accumulation.

Item Type:	Article
Uncontrolled Keywords:	Humans, Cystic Fibrosis, Pulmonary Disease, Chronic Obstructive, Inflammation, Cystic Fibrosis Transmembrane Conductance Regulator, Ion Transport
Divisions:	Faculty of Health and Life Sciences Faculty of Health and Life Sciences > Institute of Life Courses and Medical Sciences
Depositing User:	Symplectic Admin
Date Deposited:	17 May 2022 13:41
Last Modified:	18 Jan 2023 21:02
DOI:	10.1164/rccm.202109-2064tr
Open Access URL:	https://www.atsjournals.org/doi/10.1164/rccm.20210...
Related URLs:	Author Publisher
URI:	https://livrepository.liverpool.ac.uk/id/eprint/3154992