Cystic fibrosis lung disease: Current perspectives
Allergy, Asthma & Respiratory Disease
;
: 3-8, 2020.
Article
in Korean
| WPRIM
| ID: wpr-785354
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pancreas
/
Mortality
/
Chloride Channels
/
Cystic Fibrosis Transmembrane Conductance Regulator
/
Models, Animal
/
Cystic Fibrosis
/
Airway Obstruction
/
Epithelial Cells
/
Epithelial Sodium Channels
/
Half-Life
Type of study:
Prognostic study
Language:
Korean
Journal:
Allergy, Asthma & Respiratory Disease
Year:
2020
Type:
Article
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