Long-Term Ivacaftor in People Aged 6 Years and Older with Cystic Fibrosis with Ivacaftor-Responsive Mutations

INTRODUCTION: Mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR) affect the quantity and/or function of CFTR protein reaching the cell surface. Ivacaftor, a CFTR potentiator that enhances chloride transport, increases the channel-open probability of normal and dysfuncti...

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Detalles Bibliográficos
Autores principales: Pilewski, Joseph M., De Boeck, Kris, Nick, Jerry A., Tian, Simon, DeSouza, Cynthia, Higgins, Mark, Moss, Richard B.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Healthcare 2020
Materias:
Original Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7671954/
https://www.ncbi.nlm.nih.gov/pubmed/32965659
http://dx.doi.org/10.1007/s41030-020-00129-2

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7671954/
https://www.ncbi.nlm.nih.gov/pubmed/32965659
http://dx.doi.org/10.1007/s41030-020-00129-2

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