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Prolonged co-treatment with HGF sustains epithelial integrity and improves pharmacological rescue of Phe508del-CFTR

Cystic fibrosis (CF), the most common inherited disease in Caucasians, is caused by mutations in the CFTR chloride channel, the most frequent of which is Phe508del. Phe508del causes not only intracellular retention and premature degradation of the mutant CFTR protein, but also defective channel gati...

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Detalles Bibliográficos
Autores principales: Matos, Ana M., Gomes-Duarte, Andreia, Faria, Márcia, Barros, Patrícia, Jordan, Peter, Amaral, Margarida D., Matos, Paulo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6115363/
https://www.ncbi.nlm.nih.gov/pubmed/30158635
http://dx.doi.org/10.1038/s41598-018-31514-2