Functional Fluorescently Labeled Bithiazole ΔF508-CFTR Corrector Imaged in Whole Body Slices in Mice
نویسندگان
چکیده
منابع مشابه
Mechanism-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...
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ژورنال
عنوان ژورنال: Bioconjugate Chemistry
سال: 2011
ISSN: 1043-1802,1520-4812
DOI: 10.1021/bc2004457