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CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease.
Expert Rev Respir Med. 2018 Jun; 12(6):483-492.ER

Abstract

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.

Authors+Show Affiliations

a Lung Biology Group, Department of Clinical Microbiology , RCSI Education & Research Centre, Beaumont Hospital , Dublin 9 , Ireland.a Lung Biology Group, Department of Clinical Microbiology , RCSI Education & Research Centre, Beaumont Hospital , Dublin 9 , Ireland.b Department of Clinical and Biological Sciences , University of Torino , Torino , Italy.a Lung Biology Group, Department of Clinical Microbiology , RCSI Education & Research Centre, Beaumont Hospital , Dublin 9 , Ireland.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

29750581

Citation

Fernandez Fernandez, Elena, et al. "CFTR Dysfunction in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease." Expert Review of Respiratory Medicine, vol. 12, no. 6, 2018, pp. 483-492.
Fernandez Fernandez E, De Santi C, De Rose V, et al. CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease. Expert Rev Respir Med. 2018;12(6):483-492.
Fernandez Fernandez, E., De Santi, C., De Rose, V., & Greene, C. M. (2018). CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease. Expert Review of Respiratory Medicine, 12(6), 483-492. https://doi.org/10.1080/17476348.2018.1475235
Fernandez Fernandez E, et al. CFTR Dysfunction in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease. Expert Rev Respir Med. 2018;12(6):483-492. PubMed PMID: 29750581.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease. AU - Fernandez Fernandez,Elena, AU - De Santi,Chiara, AU - De Rose,Virginia, AU - Greene,Catherine M, Y1 - 2018/05/23/ PY - 2018/5/12/pubmed PY - 2019/2/23/medline PY - 2018/5/12/entrez KW - Cystic fibrosis transmembrane conductance regulator (CFTR) KW - chronic obstructive pulmonary disease (COPD) KW - cystic fibrosis (CF) SP - 483 EP - 492 JF - Expert review of respiratory medicine JO - Expert Rev Respir Med VL - 12 IS - 6 N2 - 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. SN - 1747-6356 UR - https://www.unboundmedicine.com/medline/citation/29750581/CFTR_dysfunction_in_cystic_fibrosis_and_chronic_obstructive_pulmonary_disease_ DB - PRIME DP - Unbound Medicine ER -