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Tgf-β1 Inhibits Cftr Biogenesis and Prevents Functional Rescue of ΔF508-Cftr in Primary Differentiated Human Bronchial Epithelial Cells

CFTR is an integral transmembrane glycoprotein and a cAMP-activated Cl(−) channel. Mutations in the CFTR gene lead to Cystic Fibrosis (CF)–an autosomal recessive disease with majority of the morbidity and mortality resulting from airway infection, inflammation, and fibrosis. The most common disease-...

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Detalles Bibliográficos
Autores principales: Snodgrass, Steven M., Cihil, Kristine M., Cornuet, Pamela K., Myerburg, Michael M., Swiatecka-Urban, Agnieszka
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3650079/
https://www.ncbi.nlm.nih.gov/pubmed/23671668
http://dx.doi.org/10.1371/journal.pone.0063167