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Partial Rescue of F508del-CFTR Stability and Trafficking Defects by Double Corrector Treatment

Deletion of phenylalanine at position 508 (F508del) in the CFTR chloride channel is the most frequent mutation in cystic fibrosis (CF) patients. F508del impairs the stability and folding of the CFTR protein, thus resulting in mistrafficking and premature degradation. F508del-CFTR defects can be over...

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Detalles Bibliográficos
Autores principales: Capurro, Valeria, Tomati, Valeria, Sondo, Elvira, Renda, Mario, Borrelli, Anna, Pastorino, Cristina, Guidone, Daniela, Venturini, Arianna, Giraudo, Alessandro, Mandrup Bertozzi, Sine, Musante, Ilaria, Bertozzi, Fabio, Bandiera, Tiziano, Zara, Federico, Galietta, Luis J. V., Pedemonte, Nicoletta
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8156943/
https://www.ncbi.nlm.nih.gov/pubmed/34067708
http://dx.doi.org/10.3390/ijms22105262