In the clinic


Acquired cystic fibrosis transmembrane conductance regulator protein (CFTR) dysfunction and cigarette smoking

Jonathan Kopel BS

ABSTRACT

Cystic fibrosis (CF) remains a prevalent genetically inherited disease in Caucasian populations. Investigation of the respiratory symptoms which occur in patients with CF helps us understand the pathophysiology of chronic lung disease. Environmental insults, such as cigarette smoke, can reduce the cystic fibrosis transmembrane receptor (CFTR) function or expression leading to an acquired CF phenotype and could contribute to the development and progression of smoking-related lung disease. However, it is uncertain if the acquired CF phenotype can be diagnosed with the same methods, such as the sweat chloride test and the measurement of nasal potential difference, used for genetically-acquired CF. More studies are needed to investigate the prevalence of acquired CFTR dysfunction and the differences between acquired and genetically-inherited CFTR dysfunction. Overall, acquired CFTR dysfunction challenges the distinction between genetic and acquired disorders, suggesting that environmental agents may modulate the functions of genes and the increase risk for pulmonary disease.

Keywords: Cystic fibrosis, acquired cystic fibrosis, smoking, mucociliary clearance

Article citation: Kopel J. Acquired cystic fibrosis transmembrane conductance regulator protein (CFTR) dysfunction and cigarette smoking. The Southwest Respiratory and Critical Care Chronicles 2019;7(30):43–46

From: The School of Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas

Submitted: 3/9/2019

Accepted: 6/19/2019

Reviewer: Adaobi Kanu MD

Conflicts of interest: none

This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.