title: Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis and Other Diseases of Mucus Clearance.
abstract: Chronic obstructive pulmonary disease (COPD) is a major public health problem. No therapies alter the natural history of the disease. Chronic bronchitis is perhaps the most clinically troublesome phenotype. Emerging data strongly suggest that cigarette smoke and its components can lead to acquired cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction. Findings in vitro, in animal models, and in smokers with and without COPD also show acquired CFTR dysfunction, which is associated with chronic bronchitis. This abnormality is also present in extrapulmonary organs, suggesting that CFTR dysfunction may contribute to smoking-related systemic diseases.
authors: Department of Medicine, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA; Department of Cell Developmental and Integrative Biology, The Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA.
authors: S Vamsee
authors: SV
authors: Raju
authors: Department of Medicine, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA.
authors: George M
authors: GM
authors: Solomon
authors: Department of Medicine, The UAB Lung Health Center, University of Alabama at Birmingham, Birmingham, AL, USA.
authors: Mark T
authors: MT
authors: Dransfield
authors: Department of Medicine, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA; Department of Cell Developmental and Integrative Biology, The Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA; Department of Pediatrics, The Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA. Electronic address: smrowe@uab.edu.
authors: Steven M
authors: SM
authors: Rowe
mesh_terms: D029481:Bronchitis, Chronic / Q000378:metabolism*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism*; D006801:Humans; D009079:Mucociliary Clearance; D029424:Pulmonary Disease, Chronic Obstructive / Q000378:metabolism*; D012906:Smoke
chemical_list: D012906:Smoke; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: Chronic bronchitis; Chronic obstructive pulmonary disease; Cystic fibrosis transmembrane conductance regulator; Mucociliary clearance