Article
The ΔF508-CFTR mutation inhibits wild-type CFTR processing and function when co-expressed in human airway epithelia and in mouse nasal mucosa.
BMC physiology - 24 Sept 2012
Tucker Torry A, Fortenberry James A, Zsembery Akos, Schwiebert Lisa M, Schwiebert Erik M
Abstract excerpt
BACKGROUND: Rescue or correction of CFTR function in native epithelia is the ultimate goal of CF therapeutics development. Wild-type (WT) CFTR introduction and replacement is also of particular interest. Such therapies may be complicated by possible CFTR self-assembly into an oligomer or multimer. RESULTS: Surprisingly, functional CFTR assays in native airway epithelia showed that the most common CFTR mutant,...
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