Web13 apr. 2012 · Mutations in the gene-encoding cystic fibrosis transmembrane conductance regulator (CFTR) cause defective transepithelial transport of chloride (Cl−) ions and fluid, thereby becoming responsible for the onset of cystic fibrosis (CF). One strategy to reduce the pathophysiology associated with CF is to increase Cl− transport through alternative … WebCystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J. Pediatr. 155(Suppl. 6), S73–S93 (2009). 11 De Boeck C, Cuppens H. Ion channel regulators for the treatment of cystic fibrosis. Therapy 8(6), 661–670 (2011). 12 Sagel SD, Chmiel JF, Konstan MW. Sputum Bobadilla JL, Macek M JR, Fine JP ...
Ion channels as targets to treat cystic fibrosis lung disease
WebIon channels are protein molecules that span across the cell membrane allowing the passage of ions from one side of the membrane to the other. They have an aqueous … Web29 jan. 2024 · The inflammatory airway disease cystic fibrosis (CF) is characterized by airway obstruction due to mucus hypersecretion, airway plugging, and bronchoconstriction. The cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel is dysfunctional in CF, leading to defects in epithelial transport. Although CF pathogenesis … byerly dentist blue ash
Targeting ion channels in cystic fibrosis
Web1 sep. 2015 · Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause a characteristic defect in epithelial ion transport that plays a central … Web5 okt. 2005 · Abstract. Cystic fibrosis (CF) is an autosomal recessive disorder of Cl − and Na + transport. The vast majority of CF patients have deleterious mutations in an epithelial Cl − channel called the CF transmembrane conductance regulator (CFTR). In contrast, defects in the epithelial Na + channel (SCNN1) have been associated with phenotypes … WebIon channels as targets to treat cystic fibrosis lung disease . Epithelial sodium channel Ecology Membrane Ion channel Biochemistry Medicine Biology Cell biology Pharmacology Ion transporter Mucociliary clearance Internal medicine Chloride channel Potentiator Receptor Organic chemistry Chemistry Immunology Sodium Inflammation Cystic … byerly dentistry