Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells

BACKGROUND: Mutations in the cystic fibrosis transmembrane regulator (CFTR) gene can reduce function of the CFTR ion channel activity and impair cellular chloride secretion. The gold standard method to assess CFTR function of ion transport using the Ussing chamber requires a high number of airway ep...

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Detalles Bibliográficos
Autores principales: Sutanto, Erika N., Scaffidi, Amelia, Garratt, Luke W., Looi, Kevin, Foo, Clara J., Tessari, Michela A., Janssen, Richard A., Fischer, David F., Stick, Stephen M., Kicic, Anthony
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2018
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5779693/
https://www.ncbi.nlm.nih.gov/pubmed/29360847
http://dx.doi.org/10.1371/journal.pone.0191618

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5779693/
https://www.ncbi.nlm.nih.gov/pubmed/29360847
http://dx.doi.org/10.1371/journal.pone.0191618

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