Cystic fibrosis lung disease: Current perspectives
Allergy, Asthma & Respiratory Disease
; : 3-8, 2020.
Article
Dans Ko
| WPRIM
| ID: wpr-785354
Responsable en Bibliothèque :
WPRO
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.
Texte intégral:
1
Indice:
WPRIM
Sujet Principal:
Pancréas
/
Mortalité
/
Canaux chlorure
/
Protéine CFTR
/
Modèles animaux
/
Mucoviscidose
/
Obstruction des voies aériennes
/
Cellules épithéliales
/
Canaux sodium épithéliaux
/
Période
Type d'étude:
Prognostic_studies
langue:
Ko
Texte intégral:
Allergy, Asthma & Respiratory Disease
Année:
2020
Type:
Article