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The ΔF508-CFTR mutation inhibits wild-type CFTR processing and function when co-expressed in human airway epithelia and in mouse nasal mucosa

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. R...

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Detalles Bibliográficos
Autores principales: Tucker, Torry A, Fortenberry, James A, Zsembery, Akos, Schwiebert, Lisa M, Schwiebert, Erik M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3507716/
https://www.ncbi.nlm.nih.gov/pubmed/22999299
http://dx.doi.org/10.1186/1472-6793-12-12