CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease. (3rd June 2018)

• Record Type: Journal Article
• Authors: Fernandez Fernandez, Elena; De Santi, Chiara; De Rose, Virginia; Greene, Catherine M.
• Is Part Of: Expert review of respiratory medicine. Volume 12:Number 6(2018)
• Page Start: 483
• Page End: 492
• Publication Date: 2018-06-03
• Subjects: Cystic fibrosis transmembrane conductance regulator (CFTR) -- cystic fibrosis (CF) -- chronic obstructive pulmonary disease (COPD); Respiratory organs -- Diseases -- Periodicals; Respiratory organs -- Diseases -- Treatment -- Periodicals; 616.2005
• DOI: 10.1080/17476348.2018.1475235
• Languages: English
• ISSNs: 1747-6348

Abstract

ABSTRACT: Introduction : Obstructive lung diseases such as cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) are causes of high morbidity and mortality worldwide. CF is a multiorgan genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and is characterized by progressive chronic obstructive lung disease. Most cases of COPD are a result of noxious particles, mainly cigarette smoke but also other environmental pollutants. Areas covered : Although the pathogenesis and pathophysiology of CF and COPD differ, they do share key phenotypic features and because of these similarities there is great interest in exploring common mechanisms and/or factors affected by CFTR mutations and environmental insults involved in COPD. Various molecular, cellular and clinical studies have confirmed that CFTR protein dysfunction is common in both the CF and COPD airways. This review provides an update of our understanding of the role of dysfunctional CFTR in both respiratory diseases. Expert commentary : Drugs developed for people with CF to improve mutant CFTR function and enhance CFTR ion channel activity might also be beneficial in patients with COPD. A move toward personalized therapy using, for example, microRNA modulators in conjunction with CFTR potentiators or correctors, could enhance treatment of both diseases.