Sinonasal respiratory epithelium is a highly regulated barrier that employs mucociliary clearance(MCC)as the airways first line of defense.The biological properties of the airway surface liquid(ASL),combined with coor...Sinonasal respiratory epithelium is a highly regulated barrier that employs mucociliary clearance(MCC)as the airways first line of defense.The biological properties of the airway surface liquid(ASL),combined with coordinated ciliary beating,are critical components of the mucociliary apparatus.The ASL volume and viscosity is modulated,in part,by the cystic fibrosis transmembrane conductance regulator(CFTR).The CFTR is an anion transporter of chloride(Cl-)and bicarbonate(HCO 3-)that is located on the apical surface of respiratory epithelium and exocrine glandular epithelium.Improved understanding of how dysfunction or deficiency of CFTR influences the disease process in both genetically defined cystic fibrosis(CF)and acquired conditions has provided further insight into potential avenues of treatment.This review discusses the latest data regarding acquired CFTR deficiency and use of CFTR specific treatment strategies for CRS and other chronic airway diseases.展开更多
基金This work was supported by National Institutes of Health(NIH)/National Heart,Lung,and Blood Institute(1 R01 HL133006-03)National Institute of Diabetes and Digestive and Kidney Diseases(5P30DK072482-04,CF Research Center Pilot Award)to B.A.W.,and John W.Kirklin Research and Education Foundation Fellowship Award and UAB Faculty Development Research Award to D.Y.C.
文摘Sinonasal respiratory epithelium is a highly regulated barrier that employs mucociliary clearance(MCC)as the airways first line of defense.The biological properties of the airway surface liquid(ASL),combined with coordinated ciliary beating,are critical components of the mucociliary apparatus.The ASL volume and viscosity is modulated,in part,by the cystic fibrosis transmembrane conductance regulator(CFTR).The CFTR is an anion transporter of chloride(Cl-)and bicarbonate(HCO 3-)that is located on the apical surface of respiratory epithelium and exocrine glandular epithelium.Improved understanding of how dysfunction or deficiency of CFTR influences the disease process in both genetically defined cystic fibrosis(CF)and acquired conditions has provided further insight into potential avenues of treatment.This review discusses the latest data regarding acquired CFTR deficiency and use of CFTR specific treatment strategies for CRS and other chronic airway diseases.
