Full Rescue of F508del-CFTR Processing and Function by CFTR Modulators Can Be Achieved by Removal of Two Regulatory Regions

Cystic Fibrosis (CF) is caused by mutations in the CF Transmembrane conductance Regulator (CFTR), the only ATP-binding cassette (ABC) transporter functioning as a channel. Unique to CFTR is a regulatory domain which includes a highly conformationally dynamic region—the regulatory extension (RE). The...

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Detalles Bibliográficos
Autores principales: Uliyakina, Inna, Botelho, Hugo M., da Paula, Ana C., Afonso, Sara, Lobo, Miguel J., Felício, Verónica, Farinha, Carlos M., Amaral, Margarida D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7350234/
https://www.ncbi.nlm.nih.gov/pubmed/32630527
http://dx.doi.org/10.3390/ijms21124524

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7350234/
https://www.ncbi.nlm.nih.gov/pubmed/32630527
http://dx.doi.org/10.3390/ijms21124524

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