Allergy Asthma Respir Dis.  2020 Jan;8(1):3-8. 10.4168/aard.2020.8.1.3.

Cystic fibrosis lung disease: Current perspectives

Affiliations
  • 1Department of Pediatrics, Dong-A University College of Medicine, Busan, Korea. jina1477@dau.ac.kr

Abstract

Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). These mutations alter the synthesis, processing, function, or half-life of CFTR, the main chloride channel expressed in the apical membrane of epithelial cells in the airway, intestine, pancreas, and reproductive tract. Lung disease is the most critical manifestation of CF. It is characterized by airway obstruction, infection, and inflammation that lead to fatal tissue destruction, which causes most CF morbidity and mortality. This article reviews the pathophysiology of CF, recent animal models, and current treatment of CF.

Keyword

Cystic fibrosis; Cystic fibrosis transmembrane conductance regulator; Chloride-bicarbonate transport; Epithelial sodium channel

MeSH Terms

Airway Obstruction
Chloride Channels
Cystic Fibrosis Transmembrane Conductance Regulator
Cystic Fibrosis*
Epithelial Cells
Epithelial Sodium Channels
Half-Life
Inflammation
Intestines
Lung Diseases*
Lung*
Membranes
Models, Animal
Mortality
Pancreas
Chloride Channels
Cystic Fibrosis Transmembrane Conductance Regulator
Epithelial Sodium Channels
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