64* Correction o ΔF508-CFTR in human airway epithelia and ex vivo rectal biopsies by s-cis-locked bithiazole corrector-29
نویسندگان
چکیده
منابع مشابه
Anchored PDE4 regulates chloride conductance in wild-type and ΔF508-CFTR human airway epithelia.
Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) that impair its expression and/or chloride channel function. Here, we provide evidence that type 4 cyclic nucleotide phosphodiesterases (PDE4s) are critical regulators of the cAMP/PKA-dependent activation of CFTR in primary human bronchial epithelial cells. In non-CF c...
متن کاملMechanism-based corrector combination restores ΔF508-CFTR folding and function.
The most common cystic fibrosis mutation, ΔF508 in nucleotide binding domain 1 (NBD1), impairs cystic fibrosis transmembrane conductance regulator (CFTR)-coupled domain folding, plasma membrane expression, function and stability. VX-809, a promising investigational corrector of ΔF508-CFTR misprocessing, has limited clinical benefit and an incompletely understood mechanism, hampering drug develo...
متن کاملRegulation of endogenous ENaC functional expression by CFTR and ΔF508-CFTR in airway epithelial cells.
The functional expression of the epithelial sodium channel (ENaC) appears elevated in cystic fibrosis (CF) airway epithelia, but the mechanism by which this occurs is not clear. We tested the hypothesis that the cystic fibrosis transmembrane conductance regulator (CFTR) alters the trafficking of endogenously expressed human ENaC in the CFBE41o⁻ model of CF bronchial epithelia. Functional expres...
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Traditional pulmonary therapies for cystic fibrosis (CF) target the downstream effects of CF transmembrane conductance regulator (CFTR) dysfunction (the cause of CF). Use of one such therapy, β-adrenergic bronchodilators (such as albuterol), is nearly universal for airway clearance. Conversely, novel modulator therapies restore function to select mutant CFTR proteins, offering a disease-modifyi...
متن کاملCFTR-NHERF2-LPA2 Complex in the Airway and Gut Epithelia
The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP- and cGMP-regulated chloride (Cl-) and bicarbonate (HCO₃-) channel localized primarily at the apical plasma membrane of epithelial cells lining the airway, gut and exocrine glands, where it is responsible for transepithelial salt and water transport. Several human diseases are associated with altered CFTR channel function....
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ژورنال
عنوان ژورنال: Journal of Cystic Fibrosis
سال: 2011
ISSN: 1569-1993
DOI: 10.1016/s1569-1993(11)60083-0